Mcq of Hematology

ABDULRAHMAN ABDULRAHMAN BASHIRE

CHILDREN HOSPITAL -- BENGHAZI BENGHAZI

1. The serum serum iron is often often raise raised d in the follo following wing condition condition::A. Anaemi Anaemiaa of chroni chronicc disorde disorders. rs. B. Beta Beta thal thalass assemi emia. a. C. Iron deficien deficiency cy ana anaemi emia. a. D. Sider Siderobla oblasti sticc anaemi anaemia. a. E. Congen Congenital ital spheroc spherocy ytosis tosis

Ans: -BD 2. All of the follow following ing statem statements ents are true true Exce Except: pt: A. Approximat Approximately ely 10% of RBCs RBCs are normally removed removed each day and and replaced by the the marrow to maintain the RBC count B. When the the capacity of the heme-bind heme-binding ing proteins proteins in the plasma is exceeded, exceeded, free hemoglobin hemoglobin appears in the plasma C. The most most common common cause of aplastic aplastic crisis crisis is parvovirus parvovirus B19 B19 infection infection D. The marrow marrow can increase increase its output output of RBCs RBCs two- to threefold threefold acutely acutely E. The usual marrow marrow response response to a chronic chronic hemolytic hemolytic anemia anemia is reflected by a reticulocyte reticulocyte index of 3-4 Ans:- A Normal RBC survival time is 110–120 110–120 days (half-life, 55–60 days), and approximately approximately 1% of RBCs (the senescent ones) are removed each day and replaced by the marrow to maintain the RBC count. 3. All of the follo following wing state statement mentss are tru truee Except: Except: A. The anatomic anatomic site of hematopoiesis hematopoiesis changes changes during gestati gestation on and the population population of cells cells generated at those sites are distinct B. Few neutrophi neutrophils ls are found found in the fetal circulation circulation until until the the third trimester trimester C. Thrombopoi Thrombopoietin etin is the physiologic physiologic regulator regulator of platelet platelet production production but does not act as a potent stimulator of all stages of megakaryocyte growth and development D. Erythropoi Erythropoiesis esis in utero is controlled controlled by erythroid erythroid growth factors factors produced produced solely by the fetus E. Some HbA HbA can be detected detected in even the smallest smallest embryos embryos Ans:- C Thrombopoietin (TPO) is the physiologic regulator of platelet production and acts a s a potent stimulator of all stages of megakaryocyte megakaryocyte growth gro wth and development. development. 4. Re Rega garrdi ding ng pla plate tele lets ts:: A. They have have a life span of approxima approximately tely 10 10 days B. Platelet Platelet production production is is controlled controlled by specific specific cytokines cytokines C. Platelets Platelets should should be be stored between between 2 and and 6 degrees degrees C D. Platelets Platelets can be be stored for for a maximum maximum of 48 hours hours E. A pool of platelets platelets transfused transfused can produce produce an incremen incrementt of 50 000 / ml if platelet platelet consump consumption tion is not an issue Ans:-AB Platelets are produced from megakaryocytes, megakaryocytes, which are regulated re gulated by thrombopoietin, a specific cytokine. They have a lifespan of 10-12 days thereafter being destroyed in the spleen. Platelet concentrates concentrate s should be stored at around 20 degrees C and the pH kept between 6.2 and 7.8 - these conditions reduce the risk of a change in morphology morphology of the platelets. They should also be continuously agitated to encourage gas exchange. One pool of platelets gives on average, an increment of 10 000 / ml. 5. All of the following following statements statements regarding iron deficiency deficiency are true true Except::Except::A. Because absorption of dietary iron iron is assumed assumed to be be about 10%, a diet diet containing 80-100 mg mg of iron daily is necessary for optimal o ptimal nutrition



B. Intense exercise exercise conditioning may result in iron depletion in adolescent girls C. Iron deficiency deficiency may may have effects on neurol neurologic ogic and intellectual intellectual function function D. The level level of serum ferritin provides provides a relatively accurate accurate estimate estimate of body iron stores in in the absence of inflammatory disease E. The red cell distribution distribution width width (RDW) (RDW) is elevated in iron iron deficiency deficiency but not in α and β thalassemia trait Ans:-A 6. The foll followin owing g is true true of iron defic deficienc iency y anaemia:anaemia:A. Is the commonest commonest cause of microcytic microcytic hypoch hypochromic romic anaemia anaemia in infancy infancy B. Serum transferrin transferrin is the most most reliable indicator indicator other other than BM aspiration aspiration,, of total iron stores. C. Is commonly commonly present present from 2 months months of age age upward in in term infant. infant. D. The ratio of MCV MCV to the red blood blood cell count count is useful useful in differentiatio differentiation n from thalassemia. thalassemia. E. Decreased Decreased T cell function function and and cell mediated mediated immuni immunity ty is reported. reported. Ans:ADE Low birthweight and unusual perinatal hemorrhage are associated with decreases in neonatal hemoglobin mass and stores of iron. As the t he high hemoglobin hemoglobin concentration of the newborn infant falls during t he first 2– 3 mo of life, considerable iron is reclaimed and stored. These reclaimed stores usually are sufficient for blood formation in the first first 6–9 mo of life in term infants. In low-birthweight infants or those with perinatal blood loss, stored iron may be depleted depleted earlier, and dietary dietar y sources become of paramount paramount importance. 7. A 2 year year old old Pakistani Pakistani boy has a haemoglobin haemoglobin of of 8g/dl and an MCV of 65. 65. The foll following owing tests are essential: A. Serum Serum ferritin erritin B. Serum rum B12 C. Seru Serum m folat olatee D. Faec Faecal al occul occultt blood blood E. Haemogl Haemoglobin obin electrop electropho horesi resiss Ans:-ADE Comments: In progressive iron deficiency, a sequence of biochemical and hae matologic events occurs. First, the tissue iron stores represented by bone marrow marrow haem haemosiderin osiderin disa disappear ppear . The level of serum of serum ferritin, ferritin, an iron-storage protein, provides a relatively accurate estimate of body iron stores in the absence absence of inflammatory disease. Normal ranges are age dependent, and decreased levels accompany iron deficiency. deficiency. Next, there is a decrease in serum iron (also age dependent), the iron-binding capacity of the serum (serum transferrin) increas es, and the percent saturation (transferrin saturation) falls below normal (also varies with age). When the availability of iron becomes rate limiting for haemoglobin synthesis, synthesis, a moderate accumulation of heme precursors, free erythrocyte protoporphyrins (FEP), results. results . As the deficiency progresses, progre sses, the red blood cells (RBCs) become smaller than normal and their haemoglobin content decre ases. The morphologic characteristics of RBCs are best quantified by the determination of mean corpuscular corpuscular haemoglobin (MCH) and mean corpuscular volume (MCV). Developmental changes in MCV require the use of age-related standards for diagnosis of microcytosis . With increasing deficiency the RBCs become deformed and misshapen and present characteristic microcytosis, microc ytosis, hypochromia, hypochromia, poikilocytosis and increased red cell ce ll distribution width (RDW). Iron deficiency is much commoner in more economically deprived communities, with up to 50% being affected in some inner city c ity areas.Detailed investigation is therefore there fore unnecessary in this child, unless he fails to respond to a trial of iron therapy. 8. Ir Iron on def defici icienc ency y anae anaemia mia;;A. Is characteriz characterized ed by low low serum iron and and low low TIBC B. Is associa associated ted with pica pica C. Is prevented prevented by by the the early introduc introduction tion of cow's milk milk D. Never Never require require treatment treatment under under the age age of 6 months months E. In childhood childhood is is associated associated with with chronic chronic blood blood loss in in most most cases

Ans:- B



B. Intense exercise exercise conditioning may result in iron depletion in adolescent girls C. Iron deficiency deficiency may may have effects on neurol neurologic ogic and intellectual intellectual function function D. The level level of serum ferritin provides provides a relatively accurate accurate estimate estimate of body iron stores in in the absence of inflammatory disease E. The red cell distribution distribution width width (RDW) (RDW) is elevated in iron iron deficiency deficiency but not in α and β thalassemia trait Ans:-A 6. The foll followin owing g is true true of iron defic deficienc iency y anaemia:anaemia:A. Is the commonest commonest cause of microcytic microcytic hypoch hypochromic romic anaemia anaemia in infancy infancy B. Serum transferrin transferrin is the most most reliable indicator indicator other other than BM aspiration aspiration,, of total iron stores. C. Is commonly commonly present present from 2 months months of age age upward in in term infant. infant. D. The ratio of MCV MCV to the red blood blood cell count count is useful useful in differentiatio differentiation n from thalassemia. thalassemia. E. Decreased Decreased T cell function function and and cell mediated mediated immuni immunity ty is reported. reported. Ans:ADE Low birthweight and unusual perinatal hemorrhage are associated with decreases in neonatal hemoglobin mass and stores of iron. As the t he high hemoglobin hemoglobin concentration of the newborn infant falls during t he first 2– 3 mo of life, considerable iron is reclaimed and stored. These reclaimed stores usually are sufficient for blood formation in the first first 6–9 mo of life in term infants. In low-birthweight infants or those with perinatal blood loss, stored iron may be depleted depleted earlier, and dietary dietar y sources become of paramount paramount importance. 7. A 2 year year old old Pakistani Pakistani boy has a haemoglobin haemoglobin of of 8g/dl and an MCV of 65. 65. The foll following owing tests are essential: A. Serum Serum ferritin erritin B. Serum rum B12 C. Seru Serum m folat olatee D. Faec Faecal al occul occultt blood blood E. Haemogl Haemoglobin obin electrop electropho horesi resiss Ans:-ADE Comments: In progressive iron deficiency, a sequence of biochemical and hae matologic events occurs. First, the tissue iron stores represented by bone marrow marrow haem haemosiderin osiderin disa disappear ppear . The level of serum of serum ferritin, ferritin, an iron-storage protein, provides a relatively accurate estimate of body iron stores in the absence absence of inflammatory disease. Normal ranges are age dependent, and decreased levels accompany iron deficiency. deficiency. Next, there is a decrease in serum iron (also age dependent), the iron-binding capacity of the serum (serum transferrin) increas es, and the percent saturation (transferrin saturation) falls below normal (also varies with age). When the availability of iron becomes rate limiting for haemoglobin synthesis, synthesis, a moderate accumulation of heme precursors, free erythrocyte protoporphyrins (FEP), results. results . As the deficiency progresses, progre sses, the red blood cells (RBCs) become smaller than normal and their haemoglobin content decre ases. The morphologic characteristics of RBCs are best quantified by the determination of mean corpuscular corpuscular haemoglobin (MCH) and mean corpuscular volume (MCV). Developmental changes in MCV require the use of age-related standards for diagnosis of microcytosis . With increasing deficiency the RBCs become deformed and misshapen and present characteristic microcytosis, microc ytosis, hypochromia, hypochromia, poikilocytosis and increased red cell ce ll distribution width (RDW). Iron deficiency is much commoner in more economically deprived communities, with up to 50% being affected in some inner city c ity areas.Detailed investigation is therefore there fore unnecessary in this child, unless he fails to respond to a trial of iron therapy. 8. Ir Iron on def defici icienc ency y anae anaemia mia;;A. Is characteriz characterized ed by low low serum iron and and low low TIBC B. Is associa associated ted with pica pica C. Is prevented prevented by by the the early introduc introduction tion of cow's milk milk D. Never Never require require treatment treatment under under the age age of 6 months months E. In childhood childhood is is associated associated with with chronic chronic blood blood loss in in most most cases

Ans:- B



9. The foll followin owing g cause cause a micr microcyt ocytic ic anaemi anaemia: a: A. Ulcer Ulcerativ ativee colitis. colitis. B. Perni Pernici cious ous anaem anaemia ia.. C. Methotre Methotrexate xate therapy therapy.. D. Folate Folate defici deficiency ency.. E. ß-Th ß-Thal alas assa saemi emiaa

Ans:-AE 10. Iron deficiency anaemia A. May be be prevented prevented by the the promotion promotion of milk milk intake. intake. B. Contribute to to more significant significant anaemia in infants who who are breast breast rather than formula fed fed for the first 6 months. C. Is associated associated with highe higherr incidence incidence of early early learning learning problem problems. s. D. Is confirmed confirmed by demonstrati demonstrating ng elevation elevation of serum serum ferritin ferritin conc. conc. E. Increas Increased ed suscepti susceptibil bility ity to infecti infection. on. Ans:-CE Iron is absorbed 2 to 3 times more efficiently from human milk than from cow's milk. Breast-fed infants may, therefore, require less iron from other foods. 11. The following are predisposing predisposing factors factors for iron deficiency anaemia: anaemia: A. Drinking Drinking unmodified unmodified cow's milk milk B. Prem Premat atur uriity C. Infant Infant of dia diabe betic tic mothe mother r D. Intake Intake of bottl bottlee milk milk E. Exce Excessi ssive ve tea tea drin drinking king

Ans:-ABE Comments: The predisposing factors/causes include: Inadequate dietary intake of iron. Drinking Drinking unmodified cow's milk (doorstep milk). Prematurity and low birth weight. Food stuffs and beverages which reduce iron availability including tea. 12. Ser Serum um ferritin: ferritin: A. Is raised raised in in acute acute rheumatoi rheumatoid d arthriti arthritis. s. B. Stores Stores 95% 95% of the the body' body'ss iron. iron. C. Is a useful useful measurement measurement of iron iron storage storage in the body body.. D. Should Should be measured measured in all cases of suspected suspected iron deficien deficiency. cy. E. Is incre increase ased d in hepatom hepatoma. a.

Ans:-AC Comments: Iron is an essential component co mponent in the structure of haemoglobin and myoglobin myoglobin for oxygen and carbon dioxide transport. It is also found in oxidative enzymes, c ytochrome ytochrome C and catalase. cata lase. It is absorbed in the ferrous form according to body need, aided by gastric juice and Vitamin C, and hindered by fibre, phytic acid, and steatorrhoea (about 90% of intake is excret ed in the stool). It is transported in the plasma in the t he ferric state bound to transferrin, and is stored in the liver, spleen, bone marrow and kidney kidney as ferritin ferr itin and haemosiderin. It is conserved and reused with minimal losses in the urine and sweat. In progressive iron deficiency, a sequence of biochemical and haemato logical events occurs: • First: Tissue iron stores stor es (bone marrow haemosiderin) disappear, and serum ferritin drops. Ferrit in is a relatively accurate estimate of o f body iron stores in the absence of inflammatory disease. • Next: Serum iron drops, and TIBC increases, and free er ythrocyte protoporphyrins protoporphyrins begin to accumulate. accumulate . • Later: Red cells beco become me smaller, and hypochromic with a drop in MCH and MCV. There may be poikilocytosis poikilocytosis and increased red r ed cell distribution distr ibution width. Reticulocyte Reticulocyte count cou nt may be normal or elevated, with nucleated red cells seen see n on the peripheral film. There may be thrombocytosis. The

ABDULRAHMAN BASHIRE

CHILDREN HOSPITAL -- BENGHAZI

bone marrow is hypercellular with erythroid hyperplasia. Following iron therapy there is replacement of intracellular iron enzymes and a subjective improvement within 24 hours. Within 48 hours there is a bone marrow response, with reticulocytosis evident from 2 days, and peaking at a bout 7 days. The haemoglobin level begins to increase from day 4 to day 30, and 3 months are required for complete repletion of iron stores. • In most cases of suspected iron deficiency a low Hb plus microcytosis with response to iron therapy obviates confirms the diagnosis, and there is no need to measure ferritin. 13. Regarding body iron stores:A. serum ferritin is a poor guide to iron stores B. a healthy young man will have more iron stored as ferritin than in the circulating red cell mass C. iron is the stimulant for ferritin production D. in iron deficiency stores of haemosiderin are mobilized before ferritin E. if stained with nitroprusside stain both haemosiderin and transferrin have same colour Ans:- C 14. Regarding iron deficiency anaemia: A. The commonest cause in children is chronic blood loss. B. It does not affect school progress. C. It commonly co-exists with thalassaemia. D. Cow's milk is a major source of iron for children. E. Lead poisoning is commonly associated with iron deficiency. Ans:- E Comments: Iron is absorbed in the proximal small intest ine, mediated partly by the duodenal protein mobilferrin. About 10% of dietary iron is absorbed, and iron is absorbed 2-3 times more efficient ly from human milk than from modified cow's milk. During the first years of life, because relatively small quantities of iron-rich foods are taken, it is often difficult to attain sufficiency iron. The diet should include foods such as infant cereals or formulae that have been fortified with iron. Breast fed infants s hould receive iron supplements from 4 months of age. At best, the infant is in a precarious situation with respect to iron. Should the diet become inadequate, or external blood loss occur, anaemia ensues rapidly. In children with microcytic anaemia who fail to respond to iron, thalassaemia should be considered. In this country there is an increased incidence in those from the Mediterranean and those from the Indian sub-continent. Because many such children are socioeconomically disadvantaged, there may be an associated iron deficiency anaemia. Lead poisoning in this country is usually associated with eating lead paint. Since pica is associated with iron deficiency, the two often co-exist. 15. In iron deficient anaemia A. Hypochromia, microcytosis, and reduced red cell count are typically found. B. Pencil cells may be seen in the blood film C. Koilonychia and glossitis are common complications D. Free erythrocyte protoporphyrin levels are elevated E. Ingestion of cows milk before the age of 1 year is a recognized cause

Ans;-ABDE 16. regarding A. B. C. D. E.

anaemia in childhood:Iron deficiency anaemia is the most common cause. Mucosal pallor is useful clinical sign. Macrocytic blood film indicates iron deficiency anaemia. Blood transfusions are standard treatment. Occur in less than 5% of population.

Ans :-AB Iron deficiency is indeed common; in some population has been quoted as high as 30%.

ABDULRAHMAN BASHIRE


17. The commonest presentation in child with iron deficiency anemia: A. anaemia B. Palpitation C. usually asymptomatic D. poor concentration E. heart failure

Ans :-CD 18. Concerning sideroblastic anaemia: A. Ring sideroblasts are seen in the peripheral blood film. B. Pancreatic exocrine dysfunction may occur. C. It is associated with a low serum iron. D. It may occur with anti-tuberculous therapy. E. Some cases may improve with pyridoxine.

Ans:-BE Comments: The sideroblastic anaemias are a heterogeneous group of hypochromic, microcytic anaemias, probably due to abnormalities of heme metabolism. Serum iron levels are ra ised, and ring sideroblasts are seen in the bone marrow (nucleated red cells with perinuclear haemosiderin granules that represent iron-laden mitochondria). Sub-types include: • Pearson Syndrome: associated exocrine pancreatic dysfunction due to deletions in mitochondrial DNA. • X-linked recessive: associated with splenomegaly. This may be pyridoxine sensitive or pyridoxine refractory. • Acquired: various inflammatory and malignant processes, a lcoholism. 19. The following are valid association in children with hypochromic anaemia:A. Normal serum ferritin &an elevated HbA2 point to beta thalassemia minor B. Low ferritin & normal HbA2 exclude beta thalassemia minor. C. Low ferritin point to iron deficiency. D. Raised ferritin & normal hemoglobin electrophoresis point to anaemia of chronic inflammatory disorder. E. Dimorphic blood film is consistent with recent blood transfusion.

Ans:-ACDE 20. Which of the following statement regarding iron in infancy is false :A. About 4% of iron in fortified cow milk formula is absorbed by the infants B. About 10% of iron in unfortified cow milk formula is absorbed by the infants C. About 15% of iron in breast milk is absorbed by the infants D. Absorption of iron by the infant is generally greater than in adults E. Cow's milk and human milk have approximately the same iron content

Ans:- C 21. Hypochromic microcytic anaemia is seen in: A. Alpha thalassemia B. Sickle cell anemia C. Autoimmune hemolytic anemia D. Folate deficiency E. Imerslund syndrome

Ans:- A Comment:Hypochromic microcytic anemia is usually associated with : a) Fe deficiency b) Sideroblastic anemia: - Primary X-linked recessive - Secondary alcohol - Malignancy

ABDULRAHMAN BASHIRE


- Inflammation In sideroblastic anemia there is Fe, but accumulation in the RBC mitochondria results in st ippling on the peripheral smear and ring sideroblast formation in the bone marrow. Treatment is with pyridoxine (vitamin B6). c) Lead poisoning d) Beta-thalassemia (major or intermedia) e) Alpha-thalassemia f) Anemia of chronic disease g) Copper deficiency Sickle cell disease does not produce a microcytic anemia unless associated with thalassemia as in HbSb0. To differentiate between Fe deficiency and beta-t halassemia use: 9 'Discriminant function'(DF) = MCV (fl) - RBC (x10 /l) - (5 x Hb [g/dl]) - 3.4 If DF is +ve = Fe deficiency If DF is -ve = beta-thalassemia t rait Beware; this is no use in hemodilution states (i.e. post hemorrhage or in pregnancy). Megaloblastic anemia on the other hand is usually due to: a) Folate deficiency b) Vitamin B12 deficiency c) Orotic aciduria d) Pyruvate kinase deficiency

To discriminate between folate and B12 deficiency - think: -Decreased RBC folate -Hyper-segmented neutrophils -LDH All suggest folate deficiency. Imerslund syndrome is characterized by: a) Chronic mucocutaneous candidiasis b) Decreased PTH c) Proteinuria d) Decreased vitamin B12 absorption. e) congenital autosomal recessive disorder which presents in the first 2 years of life as megaloblastic anemia and failure to thrive. 22. Regarding anaemia in childhood:A. Hemoglobin concentration of 11g/dl at the age of 3 months merits investigation. B. Iron supplements should be given to all breast feeding infants for the first 6 months of life. C. Serum iron is characteristically raised and iron binding capacity reduced in chronic hemolytic anemia. D. The majority of cases of iron deficiency anaemia in the first 2 year of childhood are nutritional in origin. E. In the treatment of iron deficiency anaemia, parenteral iron therapy raises the hemoglobin faster than oral iron. Ans:-CD 23. In the anaemia of chronic infection:A. The hemoglobin is usually <8g/dl B. The TIBC is raised C. The anaemia is usually normocytic D. The serum iron is usually reduced E. Iron supplements are contraindication

ABDULRAHMAN BASHIRE

CHILDREN HOSPITAL -- BENGHAZI Ans:-CD

24. Anaemia of acute infection and chronic disease is associated with all the following except:A. Shortened red cell survival B. Impaired iron utilization C. Iron stores are usually reduced D. Impaired erythropoietin and bone marrow response E. Usually mild anaemia with normocytic or microcytic red cells in acute infection

Ans:- C 25. Regarding blood indices: A. The haemoglobin concentration of a 3 month old boy is higher than a 13 year old boy. B. The mean corpuscular haemoglobin is low in megaloblastic anaemias. C. The mean corpuscular haemoglobin concentration is low in iron deficiency anaemias. D. The reticulocyte count increases with each year of life. E. Reticulocytes are similar in size to mature red blood cells.

Ans:- C Comments: The haemoglobin at birth ranges from 13.7 - 21.1g/dl, falling to 13.0 - 20g/dl at 2 weeks of age, and to between 9.5 and 14.5g/dl at 3 months. From there it rises gradually, with normal ranges between 6 months and 6 years being 10.5 - 14.0g/dl, and between 7 and 12 years being 11.0 - 16.0g/dl. Adult normal ranges for females are12.0 - 16.0g/dl, and for males 14.0 - 18.0g/dl. The mean cell haemoglobin (MCH) is the haemoglobin divided by the red cell count. In health, this is usually between 27 and 32pg. Any disorder which reduces red cell size reduces the amount of haemoglobin in the cell, and lowers the MCH. Likewise, disorders increasing cell size raise the value. Means corpuscular haemoglobin concentration (MCHC) equals the haemoglobin divided by the haematocrit. The normal range is 30 - 35g/dl. The MCHC is thus not the number of grams of haemoglobin in 1dl of blood, but in 1dl of pure red cells without plasma. A low MCHC is usually due to iron deficiency. The reticulocyte count in cord blood is 5%, paralleling the change fr om Hb F to Hb A. From 2 weeks of age it drops to 1%, and remains at this level until adulthood. Once menses start, females have a slightly higher reticulocyte count of around 1.6%. Reticulocytes are slightly larger than mature red cells because of the little remaining nuclear material. 26. Concerning vitamin B12:A. Deficiency can cause ataxia B. Pernicious anaemia commonly occur following terminal ileal resection C. In deficiency MCV is usually normal D. Deficiency usually occur in untreated celiac disease E. Extrinsic factor promote absorption

Ans:- A 27. Juvenile pernicious anaemia is characterized by all of the following except:A. Gastric atrophy B. Concurrent endocrinopathy C. Selective IgA deficiency D. Autosomal recessive inheritance pattern E. Chronic candidiasis

Ans;28. Abnormality associated with extravascular hemolysis include:A. Increase in plasma hemoglobin B. Decrease in serum haptoglobin C. Increase in urinary hemosiderine D. Increase in serum unconjugated bilirubin E. None of the above

Ans:- D

ABDULRAHMAN BASHIRE


29. With respect to hereditary hemolytic anaemia:A. Hereditary spherocytosis is the commonest hereditary hemolytic anaemia in Northern Europe. B. Hereditary spherocytosis is confirmed by the presence of spherocytes on the blood smear and by chromosome fragility test. C. G6PD deficiency & pyruvate kinase deficiency are inherited in an autosomal recessive manner. D. G6PD deficiency is confirmed by assaying the red cell G6PD enzymes. E. The Embden-Meyhof pathway is unable to function in the red cell that is deficient in G6PD. Ans:-AD 30. Vitamine E deficiency is associated with all of the following except:A. Greater prevalence in small premature infants. B. Manifestation as anaemia with an elevated reticulocyte count. C. Thrombocytosis. D. Physical manifestation characteristic of this deficiency are edema of the legs , labia,and eyelids in infants. E. Result in an elevated alpha tocopherol level. Ans:- E 31. All of the following statements regarding hereditary spherocytosis are true except : A. Individuals with hereditary spherocytosis may be asymptomatic without anemia B. The newborn with hereditary spherocytosis may present with anemia and .hperbilirubinemia severe enough to require phototherapy and exchange transfusion C. Isoimmune hemolytic anemia due to ABO incompatibility may mimic hereditary spherocytosis D. Thermal injury can cause spherocytosis E. Splenectomy does not eliminate most of the hemolysis of hereditary spherocytosis Ans:- E Because the spherocytes in hereditary spherocytosis are destroyed almost exclusively in the spleen, splenectomy eliminates most of the hemolysis associated with this disorder. After splenectomy, osmotic fragility often improves because of diminished splenic conditioning and less RBC membrane loss, and the anemia, reticulocytosis, and hyperbilirubinemia resolve. 32. Feature of hereditary spherocytosis A. Splenomegaly B. Conjugated hyperbilirubinemia C. Gallstones D. Hemolytic crises following fava bean ingestion E. X –linked inheritance

Ans:-AC 33. Hereditary spherocytosis:A. Is usually inherited in sex- linked recessive fashion. B. May necessitate exchange transfusion in the neonatal period. C. Should routinely be treated by Splenectomy in infancy. D. Can't be diagnosed in asymptomatic patient. E. May be complicated by gall stone.

Ans:-BE 34. Concerning hereditary spherocytosis: A. It is inherited as an autosomal dominant disorder. B. The red cells have increased osmotic resistance. C. Sufferers are prone to aplastic crises secondary to parvovirus infection. D. It is associated with macrocytosis. E. The cell shape returns to normal following a splenectomy.

Ans:-AC Comments:

ABDULRAHMAN BASHIRE


Hereditary spherocytosis (HS) is a common cause of haemolysis and haemolytic anaemia, with a prevalence of 1:5000 in Northern Europeans. Patients may be asymptomatic without anaemia and minimal haemolysis, or have severe haemolysis. It is autosomal dominant, though it is occasionally transmitted as AR. 25% of patients have no family history. The abnormality is in spectrin, a major component of the cytoskeleton. This results in loss of membrane without a proportionate loss of volume, so the red cells end up as s mall spheres rather than biconcave discs. There is an associated increase in cat ion permeability and transport, ATP utilisation, and glycolytic metabolism. The cells have decrea sed deformability impairing splenic passage, so the cells are prematurely destroyed. In addition to haemolysis, hypoplastic crises may be assoc iated with parvovirus infection. This may result in profoun anaemia with high output heart failure, hypoxia, cardiovascular collapse and death. In HS the haemoglobin level is usually 6 g/dl, and the reticulocyte c ount is elevated to 6-20%. The MCV is normal, but the MCHC is increased. Spherocytes have a smaller diameter than normal cells. The diagnosis is confirmed by an osmotic fragility test . Splenectomy eliminates most of the haemolysis associated with the disorder, but carries its own risks. In mild cases, folic acid 1mg/day is administered to prevent secondary folate deficiency. For those with severe anaemia, and/or hypoplastic or aplastic crises, splenectomy is recommended after the age of 5-6 years. Vaccines for pneumococcus, meningococcus and haemophilus influenzae should be g iven prior to splenectomy, and prophylactic penicillin continued for life. 35. In hereditary spherocytosis ,all of the following are characteristic except:A. It is autosomal dominant trait B. There is loss of red cell membrane surface without reduction of cell volume ,necessitating spherical shape in order to accommodate it's content C. Spherocytes are more likely to rupture than normal cells when suspended in hypotonic solution D. The MCV is increased & the MCHC is decreased E. Clinical severity tend to be relatively consistent within families although it varies widely from family to family Ans:-D 36. Hereditary spherocytosis :A. Is inherited as an autosomal dominant condition. B. May be confirmed by paper electrophoresis of whole blood. C. May cause sever jaundice in the newborn period. D. May be complicated by abrupt exacerbation of anaemia due to transient erythroid hyperplasia E. Is an indication for Splenectomy in the first 3 year of life. Ans:-ACD 37. Concerning hereditary spherocytosis:A. Presence of spherocytes in the neonatal period is diagnostic of this condition . B. Inheritance is as an autosomal dominant . C. The direct coomb's test is positive in > 50% of cases. D. The condition may present as jaundice in the neonatal period. E. Splenectomy reverses the tendency to form spherocytes. Ans:-BD 38. In child presenting with acute hemolysis due to an inherited hemolytic anaemia:A. Anaemia is present only after complete failure of haemtopoiesis. B. The plasma conjugated bilirubin level is elevated. C. Reticulocyte count will be less than 1%. D. Plasma haptoglobin will be elevated. E. There is excess urinary urobilinogen. Ans;- E 39. Characteristic of G6PD deficiency in patient include all of the following except:A. Sex linked recessive patter of inheritance B. Persistence of low grade anaemia in patient C. The most common clinical manifestation is episodic acute hemolysis usually follow infection or drug ingestion

ABDULRAHMAN BASHIRE


D. In black G6PD activity is near normal in reticulocytes and young erythrocytes E. Heinz bodies may be present only early in hemolytic episodes Ans:- B 40. Glucose-6-phosphate dehydrogenase deficiency: A. Is inherited as a sex-linked condition B. is not clinically manifest in girls C. Causes drug-induced haemolysis D. Is an indication for Splenectomy E. Is more pronounced in mature red blood cells

Comments:

Ans:-ACE

G6PDH deficiency is x linked recessive. It ca n manifest in females if there are 2 copies of the abnormal gene. Drugs which cause haemolysis include aspirin, antimalarials, antibacterials and sulphonamides. Splenectomy is not helpful in the management of this condition. Young red blood cells have near normal enzymatic capacity. 41. All of the following statements regarding G6PD deficiency are true except :A. Symptoms usually develop in patients with G6PD deficiency 24-48 hr after ingesting a substance with oxidative properties B. Infection may result in hemolysis in patients with G6PD deficiency C. A pregnant woman who ingests oxidative drugs may cause hemolytic anemia in a fetus with G6PD deficiency D. Enzyme activity in affected persons is 10% of normal or less E. The usual dose of aspirin causes clinically relevant hemolysis in the A variety of G6PD deficiency Ans:- E The usual doses of aspirin and trimethoprim sulfamethoxazole do not cause clinically re levant hemolysis in the A- variety. However, aspirin administer ed in doses used for acute rheumatic fever (60– 100 mg/kg/24?hr) may produce a severe hemolytic episode. When hemolysis has occurred, supportive therapy may require blood transfusions, although recovery is the rule when the oxidant agent is removed. 42. All of the following statements about Glucose-6-phosphate dehydrogenase deficiency are false except: A. Is more common in women than in men. B. Is caused by mutations of the G6PD gene on chromosome 4. C. Often leads to chronic haemolysis. D. Requires treatment with desferrioxamine to prevent iron overload. E. Is associated with haemolytic crises induced by sulfonamides. Ans:- E G6PDH catalyses the synthesis of NADPH from the hexose monophosphate pathway. The NADPH is t hen used to reduce glutathione and hence in conjunction with glutathione reductase re duces cellular oxidative damage. G6PDH enzyme deficiency, caused by mutations in the X-linked (Xq28) G6PD gene, affects over half a billion people worldwide. It is a balanced polymorphism associated with resistance to falciparum malaria in heterozygous females. This evolutionary advantage outweighs the small negative effect of affected hemizygous males. At least 400 G6PD variants have been identified. The various mutations lead to altered expression of G6PD or an altered half-life of the enzyme. There are a number of disease manifestations: - patients may be asymptomatic - patients may present with neonatal jaundice - usually the deficiency manifests as acute haemolytic crises provoked by oxidising stress e.g. certain foods - fava bean, chemicals - naphthalene, certain drugs - dapsone, primaquine, chloroquine, aspirin

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(high doses), sulphonamides, nitrofurantoin, vitamin K and nalidixic acid. Infections can also provoke haemolysis. Haemolytic crises vary in severity, rarely chronic haemolysis may occur. If severe, G6PD deficiency can cause immunodeficiency by reducing the NADPH required for activation of the NADPH oxidase enzyme (cf. CGD). 43. Chronic intravascular haemolysis is associated with: A. Splenomegaly. B. Haemosiderinuria. C. Increased incidence of gallstones.. D. Reduced transferrin levels. E. Increased serum haptoglobins.

Ans:-BC Comments: Haemolysis is defined as the premature destruction of red cells. If the rate of destruction exceeds the capacity of the marrow to produce red cells then anaemia results. The normal red cell survival is 120 days, and 1% of red cells are removed per day and replaced by the marrow. During haemolysis, the red cell survival is shortened, and there is increased marrow activity (raised reticulocyte count). Marrow output can increase 2-3 fold acutely, and 6-8 fold in long-standing cases. In chronic haemolytic anaemia, er ythroid hyperplasia may be so extensive that the medullary spaces may expand at the expense of cortical bones (particularly skull and long bones). Intravascular haemolysis increases circulating haemoglobin which binds to haptoglobin reducing the circulating levels of it. There is an increase in urine haemoglobin and haemosiderin. Increased bilirubin production from heme results in increased faecal urobilinogin, which is reabsorbed and excreted in the urine. 44. The following drugs should be avoided in all patients with glucose-6-phosphate dehydrogenase deficiency: A. Quinine B. Nitrofurantoin C. Sulphapyridine D. Chloramphenicol E. Chloral hydrate Ans:- ABCD Comments: In glucose-6-phosphate-dehydrogenase deficiency (G-6-PD) subjects are susceptible to developing acute haemolytic anaemia on taking a number of common drugs. Ingestion of fava beans may result in haemolysis in severe cases, or when they are eaten raw. G-6-PD is genetically heterogeneous. The risk from drugs therefore varies from patient to patient. There is no test available to identify potential risk in G-6-PD deficiency. The risk of severity of haemolysis is a lmost always dose related. Drugs with a definite risk in most G-6-PD deficient subjects include: • Sulphonamides and Dapsone. • Methylene blue • Nitrofurantoin • Primiquine • Quinilones (including Ciprafloxacin, Nalidixic acid) 45. The following are associated with a definite risk of hemolysis in most glucose-6 phosphate dehydrogenase deficient subjects: A. Primaquine B. Methyllene blue C. Nalidixic acid D. Co-trimaxazole E. Nitrofurantoin Ans:- ABCDE

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glucose-6 phosphate dehydrogenase reduces NADP to NADPH. NADPH is used by glutathione reductase to reduce (oxidised) glutathion (toGSH). This replenishes the supply of reduced-SH groups in the erythrocyte membrane protecting it from oxidant stress. Deficiency of G6PD renders the red cell membrane susceptible to oxidant stress and haemolysis may r esult following exposure to an oxidising agent of the environment (drugs, fava beans, infections, DKA). G6PD also reduces NAD to NADH which is used by methaemoglobin reductase. Therefore G6PD deficiency may also result in increased oxidation of Hb (metHb) with the apperarance of Heinz bodies o n the blood film. Drugs that cause hemolysis in G6PD deficiency are oxidizing a gent. The BNF gives two lists of drugs as follows: (1)- Definite risk of hemolysis in most : *dapson. *methylene blue. *nitrofurantoin. *Primaquine. *quinolones *sulphonamides.

2)-Possible risk in some: *aspirin (high dose). *chloroquine. *menadione (vit K). * probencid. *quinidine,. *quinine

46. G6PD deficiency:A. Is inherited as sex-linked condition. B. Is not clinically manifest in girls C. Causes drug – induced hemolysis. D. Is an indication for Splenectomy. E. Is more pronounced in mature RBC

Ans:-ACE Most male show complete expression but female less so; American black the prevalence in male is 15% & in female is 2% 47. Regarding glucose-6-phosphate dehydrogenase: A. It is an important enzyme in the kreb cycle. B. Deficiency is associated with an increased susceptibility to falciparum malaria infection. C. Deficiency may be associated with chronic haemolytic anaemia. D. Deficiency is inherited as an autosomal dominant. E. Deficiency may be associated with primiquine-induced haemolysis.

Ans:- E Comments: Glucose-6-phosphase dehydrognease (G-6-PD) is one of the enzymes in the pentose phosphate pathway (Hexose Monophosphate Shunt). The most important function of this pathway is to maintain glutathione in a reduced state as protection against oxidation of the red cell. Deficiency is X-linked, and extre mely common, affecting more than 200 million people. Heterozygous females have increased res istance to falciparum malaria, outweighing the small negative effect on hemizygous males. Major symptoms include: • Episodic or induced haemolytic anaemia: there is considerable variation in the defect among different racial groups, with Mediterranean whites affected more severely than Africans. Haemolysis may precipitated by: 1. Drugs: Antibacterials: sulfonamides, septrin, nalidixic acid, chlora mphenicol, nitrofurantoin. Antimalarials: primaquine, chloroquine, quinacrine. Others: Vitamin K, methylene blue, Aspirin. 2. Chemicals: Phenylhydrazine, benzene, naphthalene. 3. Illness: Diabetic ketoacidosis (DKA), hepatitis. • Chronic haemolytic anaemia: chronic non-spherocytic haemolytic anaemia is assoc iated with profound deficiency of G-6-PD. Splenectomy is of little value in these types.

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48. Regarding autoimmune hemolytic anaemia (AIHA):A. The direct coombs test will be positive. B. It is frequently due to an intercurrent viral infection in children. C. Spherocytes are commonly seen on the blood smear. D. AIHA is divided into warm & cold types depending on whether the patient is febrile or afebrile. E. It is often associated with connective tissue disorders in the adolescent. Ans:-ABCE 49. A 17 year old Nigerian presents with moderately enlarged spleen, haemoglobin of 7.2 g/dL, a red cell count of 3.5 x 1012/L, and a reticulocyte count of 7%. The following statements are correct: A. Recent chloramphenicol administration would be of diagnostic significance B. If the Coombs' test was positive, paroxysmal nocturnal haemoglobinuria is possible diagnosis C. Homozygous thalassaemia is possible diagnosis D. If Heinz bodies are present, red cell glucose 6-phosphate dehydrogenase deficiency is likely diagnosis E. homozygous sickle cell disease is likely diagnosis. Ans:-C D Comments: b-thalassaemia major (homozygous) thalassaemia is possible diagnosis. Splenic atrophy from splenic infarcts occurs in sickle cell disease. Chloramphenicol may cause aplastic anaemia - there will be no reticulocyte response. Heinz bodies are oxidized denatured haemoglobin. During crisis of G6PD deficiency, blood film also shows contracted and fragmented cells due to oxidant stress. PNH is not an autoimmune haemolytic anaemia, hence Coombs test is negative. It is a rare acquired defect of red cell membrane making it susceptible to lysis by complement. 50. Features which may be seen on the blood film of a patient with haemolysis include: A. Polychromasia B. Howell Jolly bodies C. Elliptocytes D. Target cells E. Heinz bodies

Ans:-ABDE Comments: The following are common abnormalities of red cell morp hology: • Polychromasia: younger cells have a bluish tinge (basophilia), and are larger than average, and may contain residual nuclear material (reticulocytes). The presence of many basophilic forms in a blood film produces a multicoloured effect known as polychromasia. • Microcytosis indicates small red cells, anisocytosis variation in size, and macrocytosis a large size. Poikilocytosis indicates altered shape. • Spherocytes may indicate hereditary spherocytosis, and may also be found in acquired haemolytic anaemias, while elliptocytes are found in hereditary elliptocytosis. • Schistocytes are fragments of red ce lls, as seen in DIC or haemolytic uraemic syndrome. • In splenic dysfunction: target cells and red ce lls containing nuclear fragments known as Howell Jolly bodies are seen. Pappenheimer bodies are iron containing inclusions, and when seen together with Howell Jolly bodies suggest previous splenectomy or reduced splenic function. • Heinz bodies: are denatured haemoglobin which result from deficiencies of enzymes of the penthose phosphate pathway. This damages the red cell leading to haemolysis and removal of them by the spleen. The following conditions are haemaglobinopathies:A. G6PD deficiency. B. von Willebrand disease. C. Spherocytosis. D. Thalassaemia..

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E. Sickle cell disease. Ans:-DE 51. Homozygous beta thalassemia A. Arise from alteration in the beta globin gene on chromosome 10 . B. May present with FTT. C. Is associated with elevated HbF in infancy & older children. D. May require Desferoxamine treatment. E. Doesn't responds to iron treatment despite microcytic indices.

Ans:-BCDE 52. Which is compatible with B-thalassemia major? A. HbF 96% HbA2 4% B. Hb A1 40%, Hb F 58%, Hb A 2 2% . C. Hb A1 85%, Hb F 10%, HbA2 5%

Ans: A 53. In beta thalassaemia:A. Beta thalassaemia major presents at birth with profound anaemia B. The reticulocyte count is low C. Diagnosis can be made by Haemoglobin electrophoresis D. Anaemic patients should receive regular iron supplementation E. Splenectomy is contraindicated due to extramedullary haemopoiesis

Ans:- C Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic de ficiency in the synthesis of beta-globin chains. In the homozygous state, beta thalassemia (ie, thalassemia major) causes severe transfusion-dependent anemia with high reticulocyte count. The anaemia becomes manifest in late infancy. Diagnosis is by haemoglobin electrophoresis. Increased iron deposition resulting from multiple life-long transfusions and enhanced iron absor ption results in secondary iron overload. This overload causes clinical problems similar to those observed with primary hemachromatosis (eg, endocrine dysfunction, liver dysfunction, cardiac dysfunction) o The physical findings are related to severe anemia, ineffective erythropoiesis, extramedullary hematopoiesis, and iron overload resulting from transfusion and increased iron absorption. o Skin may show pallor from anemia and jaundice from hyperbilirubinemia. o The skull and other bones may be deformed secondary to erythroid hyperplasia with intramedullary expansion and cortical bone thinning. o Heart examination may reveal findings of cardiac failure a nd arrhythmia, related to either severe anemia or iron overload. o Abdominal examination may reveal changes in the liver, gall bladder, and spleen. Hepatomegaly related to significant extramedullary hematopoiesis typically is observed. Patients who have received blood transfusions may have hepatomegaly or chronic hepatitis due to iron overload; transfusion-associated viral hepatitis resulting in cirrhosis or portal hypertension also may be seen. The gall bladder may contain bilirubin stones formed as a result of the patient's life-long hemolytic state. Splenomegaly typically is observed as part of the e xtramedullary hematopoiesis or as a hypertrophic response related to the extravascular hemolysis. o Extremities may demonstrate skin ulceration. o Iron overload also may cause endocrine dysfunction, especially affecting the pancreas,t estes& thyroid. 54. In thalassemia:A. The diagnosis is made on blood film. B. The anaemia is due to deficiency in synthesis of globin chain. C. The anaemia is mainly due to hemolysis. D. Splenectomy is the treatment of choice at diagnosis. E. The serum iron level is normal at diagnosis.

Ans;- BE

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55. The clinical picture of B-thalassemia major:A. Present at birth. B. Is characterized by iron deficiency anaemia. C. May initially present as FTT D. Include hepatosplenomegaly. E. Is confirmed if investigation reveals markedly raised HbF on electrophoresis.

Ans:- CDE 56. Beta thalassemia major:A. Is associated with relative excess of b globin molecules. B. Result in blood film exhibiting microcytic hypochromic red cell. C. Produce generalized osteoporosis. D. Is commonly associated with reduced serum iron. E. Result in delayed puberty.

Ans:- BCE 57. The following are characteristic finding in beta thalassemia :A. Hypochromic microcytic red cells B. Low serum iron C. Cardiomyopathy. D. Presentation in the neonatal period. E. Frontal bossing of the skull.

Ans:-ACE 58. β Thalasemia major is associated with all of the following except:A. Defect in β globin chain synthesis B. In the newborn infant is clinically & hematologically normal C. The determination of HbA2 is important in diagnosis D. Onset of clinical symptoms & sign usually begin between 6-12 month of age E. Peripheral blood show hypochromic and usually microcytic anaemia

Ans;- C 59. Which one of the following doesn't characterize heterozygote β thalassemia ( minor / trait):A. In most patient anaemia & clinical symptoms are absent B. Life expectancy is shortened C. Varieties of this disorder are best distinguished by quantification of A2 & HbF D. Peripheral blood examination reveal microcytosis ,target cells , & variable degree of hypochromia E. Severity depend on the degree of suppression of β globin synthesis Ans:- B 60. All of the following statements are true Except: A. In patients who have Hb SS electrophoresis pattern and concomitant microcytosis, iron deficiency or a combination of Hb S with or thalassemia must be considered a possible diagnosis B. Febrile infants with sickle cell disease should be managed in the hospital C. The iron in hemoglobin is normally in the ferric state, which is essential for oxygen transport D. Children with homozygous β thalassemia usually become symptomatic from progressive hemolytic anemia during the second 6 mo of life if not treated E. The thalassemia traits present as microcytic anemia, which can be mistaken for iron-deficiency anemia Ans:- C 61. A black West African girl aged 15 years is complaining of severe pain in both legs. The haemoglobin is 8g/dl. Sickle cell anaemia is unlikely to be the cause of her symptoms if A. she is jaundiced B. she has haematuria C. x-ray of the spine shows osteoporosis D. menarche occured at 12 years E. the urinary osmolality is 800mosm/kg (specific gravity approximately 1022)

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CHILDREN HOSPITAL -- BENGHAZI Ans:- DE

Comments: a+b-expected in sickle cell anaemia, c-x-ray usually shows fishmouth vertebrae (infarcts), d-growth + development usually impaired, e-almost all are unable to produce concentrated urine 62. In the management of acute sickle cell painful crises: A. Patients should receive continuous oxygen and monitoring of SaO2. B. Patients should receive intravenous antibiotics. C. Intravenous opiate analgesia should be titrated until adequate control of pain. D. Blood transfusions should be given. E. Intravenous hydroxyurea should be given.

Ans:-ABC Comments: Painful episodes can often be managed with oral Paracet amol -/+ codeine. Severe episodes require hospitalisation with parenteral opiates. Anti-inflammatories may decrease o r eliminate the need for narcotics. Dehydration and/or acidosis should be rapidly corr ected by intravenous fluids. Packed cells are specifically indicated for acut e splenic sequestration and aplastic crises. The latter may require splenectomy. Intravenous antibiotics should be given to cover the possibility of haemophilus or pneumococcal infection. Chemotherapy regimens that stimulate fetal haemoglobin synthesis have been employed with beneficial effect on an experimental basis. These include hydroxyurea and hydroxybutyrate. These are given as maintenance therapy. 63. The following are recognised features of sickle cell disease: A. Dactylitis B. Frontal bossing of the skull C. Abdominal pain D. Chronic leg ulceration E. Anaemia from birth

Ans:- ACD Comments: Sickle cell anaemia is characterised by severe chronic haemolytic disease resulting from premature destruction of brittle, poorly deformable erythrocytes. Ot her manifestations are due to ischaemia resulting from vascular occlusion by masses of sickle cells. The clinical course is typically associated with crises. The manifestations vary considerably with age.  Newborns: haemolytic anaemia from 2-4 months as fetal haemoglobin is replaced by Hb S, acute sickle dactylitis (hand-foot syndrome). Pre-school: acute painful vaso-occlusion crises, affect ing extremities. School children: painful crises affecting head, chest , abdomen, back, the site being typical for an any individual patient. Episodes may be precipitated by intercurrent illness. Late changes: - infarction of bone marrow or bone. - splenic infarction between 6 and 60 months contributing to autosplenectomy. - pulmonary infarcts (acute chest syndrome). - stroke caused by cerebrovascular occlusion -/+ hemiplegia. - ischaemic damage to myocardium, liver and kidneys, with progressive impairment of renal function and concentrating ability. Spleen changes: in young children the spleen is enlarged, with occasional acute splenic sequestration. Altered splenic function increases the risk of serious infection particularly meningitis sepsis caused by pneumococci and haemophilus influenzae (polysaccharide encapsulated organisms). As the child ages, auto splenectomy reduces spleen size. Cardiomegaly is invariably present in older children (sickle-related cardiomyopathy), secondary haemosiderosis from increased iron absorption may damage liver, pancreas and heart, and there may be gal l stone formation. Puberty is frequently delayed, and chronic leg ulcers o ccur in late adolescence.

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64. A healthy, 39-week gestation male weighing 3.5 kg is born to a mother who has chronic anemia. The infant’s physical examination findings are normal, and his hematocrit is 49% (0.49). The mother asks you about the need for vitamins and iron for her newborn son. Of the following, the BEST response is that term babies need to begin iron therapy at A. birth B. 2 weeks of age C. 4 months of age D. 9 months of age E. no time Preferred Response: C Term newborns have accrued sufficient iron stores in the latter part of gestat ion to sustain them for 3 to 4 months after birth; this is true even when the mother has anemia. Although human milk contains lower quantities of iron, its bioavailability is greater and, therefore, breastfed infants do not require replacement therapy until 4 months of age. Preterm infants miss out on iron accretion in utero during the last tr imester of pregnancy and may require iron supplementation if they are taking full-volume enteral feedings as early as 2 to 4 weeks of age. Iron supplementation is not required at birth except in the rare circumstance of congenital anemia. Iron supplementation is required for normal hematopoiesis and brain growth and function, and if not provided by 6 months of age, characteristically leads t o iron deficiency anemia. 65. You are called to the delivery room to evaluate an infant who has been delivered by spontaneous vaginal delivery. The term infant weighs 3.6 kg and has some grunting respirations. You decide to observe her in the newborn intensive care unit. One hour later, you are notified that the infant is experiencing pronounced respiratory distress and oxygen saturations in the 70% range in the right hand and in the 50% range in the right foot. There is poor perfusion. There are no murmurs, but there is a pronounced precordial lift and a loud second heart sound. Of the following, the MOST likely diagnosis for this infant is A. congenital diaphragmatic hernia B. congenital toxoplasmosis C. hypoplastic left heart syndrome D. persistence of the fetal circulation E. tetralogy of Fallot Preferred Response: D Determining the cause of cyanosis and hypoxemia in the neonate is crit ically important, but it can be difficult, especially during the first few minutes of the presentation. Diagnostic considerations in these potentially critically ill infants may include persistence of the fetal circulat ion, with or without meconium aspiration, and cyanotic congenital heart disease. The neonate described in the vignette clearly is experiencing a disturbance in the process of oxygen delivery, and several clues suggest persistence of the fetal circulation. Detecting this condition requires a clear understanding of the ductus arteriosus and it s function in the transitional circulation. In the normal heart, the right atrium and right ventricle deliver desaturated blood to the organ of oxygenation. In the fetus, this organ is the placenta, and its fetal blood supply is via the umbilical arter y, which arises from the fetal descending aorta. The ductus arteriosus provides a fetal shunt ing pathway, allowing the right side of the heart to deliver desaturated blood to the placenta by shunting this blood away from the high-resistance pulmonary arteries and into t he descending aorta. This direction of flow is due in part to the slightly higher fetal pulmonary vascular resistance compared with fetal systemic vascular resistance because the lungs are filled with fluid and the placenta is a low-resistance circuit. At birth, when the lungs expand with air and the placenta is removed from the circulation, pulmonary vascular resistance falls and systemic vascular resistance increases. This leads to a reversal of flow across the ductus arteriosus (from the system into the pulmonary circuit). Over subsequent hours and days, the ductus arteriosus begins the process of spontaneous closure.

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When the pulmonary vascular resistance exceeds the systemic vascular resistance, right-to-left shunting across the ductus arteriosus persists as long as the ductus art eriosus is patent. Neonates who have severe lung disease, including pneumonia, lung collapse, and pulmonary hypoplasia, may have pulmonary vascular resistance that exceeds the systemic vascular resistance. When this occurs in the presence of a patent ductus arteriosus, there is right-to-left shunting of blood in a pattern that is similar to the fetal circulation. Hence, the term “persistence of the fetal circulation,” or perhaps bett er stated, “persistent pulmonary hypertension of the newborn.” This condition leads to highly desaturated blood entering the descending aorta, often evidenced by lower oxygen saturation in the lower body than in the upper body. When this process results from pulmonary pathology, there is likely intrapulmonary ventilation-perfusion (V-Q) mismatch as well, yielding the return of relatively desaturated blood to the left heart, which then delivers inadequately oxygenated blood to the aorta. Signs of an underlying pulmonary process may include grunting to maintain alveo li and small airway patency, tachypnea, and ultimately respiratory distress and failure, as described for the infant in the vignette. The oxygenation saturation pattern of the infant supports the diagnosis of persistence of the fetal circulation. She has no murmur because she has no turbulent blood flow in her heart, and her precordial lift results from the increased volume, pressure, and work load imposed on the right ventricle. Poor per fusion is caused by failure (diminished ventricular function) of the right ventricle and its impact on the left ventricle. Neonates who have diaphragmatic hernia often present with respiratory distress and a shift of the precordium to the right chest because the gastrointestinal contents typically occupy the left thorax. Infants who have toxoplasmosis may have signs and symptoms of systemic infection, but they are unlikely to present solely with pulmonary pathology. The hypoplastic left heart syndrome relies on right-to-left shunting at the ductus arteriosus to supply systemic blood flow, but affected infants have normal lungs and low pulmonary vascular resistance, with oxygen saturation typically in the 80% to 90% range. Tetralogy of Fallot usually presents with the murmur of pulmonary stenosis, and when the ductus arteriosus is present, blood flow is left to right because pulmonary vascular resista nce and the lungs are normal 66. In sickle cell disease:A. Dactylitis is common presentation in infancy. B. Autosplenectomy has generally occurred by the age of 6 months. C. Sickle lung is an indication for exchange transfusion. D. Aplastic crises may arise as consequence of parvovirus infection. E. Salmonella osteomyelitis occur.

Ans:-ACDE Dactylitis, often referred to as hand-foot syndrome, is frequently the 1st manifestation of pain in children with sickle cell anemia, occurring in 50% of children by 2 yr of age. Dactylitis often presents with symmetric swelling of the hands and/or feet Acute splenic sequestration is a life-threatening complication occurring primarily in infants, and may occur as early as 5 wk of age. Approximately 30% of children with sickle cell anemia have s ignificant splenic sequestration episodes; Repeated episodes of splenic sequest ration are common, occurring in approximately 50% of patients. The majority of recurrent e vents occur within 6 mo of the previous event. 67. Sickle cell disease :A. Is caused by a mutation on chromosome 16 B. Will give a positive sickle test result at birth C. Is associated with strokes in 25% of patients by the age of 45 years D. Is an indication for autologous haemopoietic stem cell transplant E. Can be co-inherited with alpha or beta-thalassaemia

Ans:- CE Sickle cell disease is an autosomal recessive condition caused by a single amino acid mutation in the Beta globin gene on chromosome 11. The alpha globin genes are coded on chromosome 16. It can be co-inherited with alpha or beta thalassaemia. Clinical effects do not become apparent until 6 to 12 months of age when

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haemoglobin switching occurs and the amount of HbF (fetal haemoglobin) decreases and the amount of HbS (sickle haemoglobin) increases. This is why the sickle test, which detects the presence of sickle haemoglobin, is often not positive until 6-9 months of age and cannot be guaranteed until one year of age. Clinical problems include strokes and these are common, occuring in 25% of patients with homozygous sickle disease by the age of 45 years. Patients with severe clinical manifestations of sickle disease have been treated with allogeneic bone marrow transplantation from an HLA-identical sibling. Autologous bone marrow transplantation is where the patients own bone marrow is replaced and would not be of benefit in this condition. 68. Sickle cell disease :A. Is associated with increased the polymerization of deoxygenated Hb B. Is recognized cause of prolonged neonatal jaundice. C. Increased the susceptibility to infection with encapsulated bacteria. D. Is cause of childhood stroke ( cerebrovascular disease ). E. Is conventionally managed by regular blood transfusion.

Ans:-ACD 69. In sickle cell disease:A. There is abnormality of red cell membrane. B. Symptoms result from tissue infarction. C. There is some protection against Plasmodium falciparum malaria. D. Sickling crises can be prevented by morphine. E. Sickling risk can be reduced by exchange transfusion.

Ans:-BE Individuals with sickle cell trait have lower levels of Plasmodium falciparum parasitemia, higher hemoglobin counts, and less severe reinfections than individuals with homozygous Hb A. 70. Regarding sickle cell anaemia :A. Positive Sickling test is diagnostic of sickle cell disease B. Roughly 5% of African are carriers of the sickle cell trait. C. The heterozygous condition is usually asymptomatic. D. Treatment with desferoxamine has significantly improved the life expectancy in sickle cell disease. E. There is increased predisposition to salmonella osteomyelitis. Ans:-CE the incidence of sickle cell trait is 7–10% of African-Americans (1 in 12. ). The amount of Hb S in individuals with sickle cell trait is <50%. 71. The mother of a baby in the postnatal ward has an antenatal screening test for sickle haemoglobin. This is positive deposite a normal Hb & MCV. The Hb-electrophoresis shows HbA 55% and Hbs 45%. Her partner has a microcytic anaemia, and Hb-electrophoresis shows an HbA band only with HbA2 quantitation of 4.8%. Which diagnosis is impossible in the baby? A. Sickle cell trait. B. Haematologically normal. C. B-thalassaemia trait. D. Sickle-B-thalassaemia. E. Sickle cell diseas(HbSS). Ans:- E The mother probably has a sickle cell tra it,the father has no evidence of Hbs & has a laboratory & Hbelectrophoretic profile compatible with B0thalassaemia trait. The baby may therefore have : *Normal Hb. *Sickle cell trait. *B-thalassaemia trait. *Sickle-b-thalassaemia.

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It is not possible for the baby to have sickle ce ll disease (HbSS). 72. Sickle cell anaemia:A. Is associated with increased risk of gallstone. B. Is associated with increased risk of nocturnal enuresis C. Can be diagnosed antenatally D. Is an indication for prophylactic penicillin E. Is associated with episodes of acute anaemia

Ans:-ABCDE 73. Complications of sickle cell disease include: A. Pneumococcal infection in the first four weeks of life. B. Stroke. C. Dactylitis in the first four weeks of life. D. Cholecystitis. E. Haematuria.

Ans:-BDE Dactylitis (swelling of the joints)is the most common presentation symptom of sickle cell disease. It usually occurs between six to twelve months of age when haemoglobin production switches from the production of gamma chains to the production of the abnormal beta chains, and may present to an orthopaedic surgeon. This means that the amount of foetal haemoglobin (which is prot ective against sickle crisis)decreases and the amount of sickle haemoglobin increases. Pneumococcal infections (and infections with other encapsulated bacter ia such as Haemophilus influenza B and neisseria meningitides) are common in patients with sickle cell disease from the age of 2 to 3 years when they have auto-infection of the spleen and are rendered hyposplenic. They should be protected against these diseases with vaccination and prophylactic antibiotics. Strokes are very common in patients with sickle c ell disease occurring in 25% of patients with homozygous sickle cell disease by the age of 45 years. Cholecystitis occurs in patients with sickle cell disease as with a haemolytic anaemia because of the production of pigment gallstones. Haematuria occurs in sickle cell disease because of renal papillary necrosis. Interestingly it can also occur in patients with sickle cell trait. 74. Which of the following statement is false regarding the pathophysiology of sickle cell disease:A. Infection is associated with increased anemia by suppression of RBC production B. Conversion of RBC from normal biconcave discs to sickle form require the deoxygenation of haemoglobin C. Hypoxiemia and acidemia promote sickling by decreasing oxygen saturation of Hb D. Dehydration doesn't promote sickling E. Sickle cell increase whole blood viscosity producing local ischemia Ans:-D 75. Which one of the following clinical manifestation isn't common to sickle cell disease:A. Vaso-occlusive crisis is more common than aplastic crisis B. Hyperhemolytic crisis is often associated with red cell G6PD deficiency C. Aplastic crisis is often associated with viral or bacterial infection D. Salmonella sepsis is commonly associated with salmonella osteomyelitis E. Hand-Foot syndrome may be initial manifestation of sickle cell disease Ans:-D 76. The following are seen in sickle-cell anaemia A. dactylitis B. retardation of secondary sexual characteristics. C. pathognomic fundal changes D. cardiac signs simulating mitral stenosis E. normal urinary concentrating ability only if the sickle-cell trait is present

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CHILDREN HOSPITAL -- BENGHAZI Ans:-ABCD

Comments: Dactylitis - inflammation of the fingers is a feature of SCD due to expansion of bone marrow. B aconsequnce of ill health with hypogonadotrophic hypogonadism. A so called black sunburst retinopathy is pathognomonic of sickle cell disease. Retinal vein thrmbosis is also seen. Poor urinary concentration is a feature of the disease but is unaffected with the trait. 77. Recognised features of sickle cell trait include: A. Moderate anaemia B. Increased risk of anaesthesia C. Reduced renal concentrating ability in adolescence D. Episodic haematuria E. Splenomegaly

Ans:-BCD Comments: Heterozygous expression of the sickle haemoglobin gene (Hb AS) is usually assoc iated with a totally benign clinical course. The haematological findings are indistinguishable from normal. About 40% of their haemoglobin consists of Hb S, and under normal circumstances this is insufficient to produce sickling. Under severe hypoxic stress, vaso-occlusive complications may occur. This may occur under anaesthesia or at high altitudes. This may result in splenic infarcts and other ischaemic sequelae. Decreased renal concentrating ability is usually present in older children and adults, and occasional gross haematuria may occur. The diagnosis is confirmed by haemoglobin electrophoresis and sickle test ing. 78. A 10 year old West African boy presents with Hb 8g/dl and pains in his legs. Sickle cell disease is unlikely if: A. He is jaundiced. B. He has gross splenomegaly. C. Puberty began at 12 years. D. His urine osmolality is 800mosmol/L, specific gravity 1022. E. There is a mid systolic murmur. Ans:-BCD Comments: Sickle cell anaemia is characterised by severe chronic haemolytic disease resulting from premature destruction of brittle, poorly deformable erythrocytes. Ot her manifestations are due to ischaemia resulting from vascular occlusion by masses of sickle cells. The clinical course is typically associated with crises. The manifestations vary considerably with age.  Newborns: haemolytic anaemia from 2-4 months as fetal haemoglobin is replaced by Hb S, acute sickle dactylitis (hand-foot syndrome). Pre-school: acute painful vaso-occlusion crises, affect ing extremities. School children: painful crises affecting head, chest, abdomen, back, the site being typical for an any individual patient. Episodes may be precipitated by intercurrent illness. Late changes: o infarction of bone marrow or bone. o splenic infarction between 6 and 60 months contributing to autosplenectomy. o pulmonary infarcts (acute chest syndrome). o stroke caused by cerebrovascular occlusion -/+ hemiplegia. o ischaemic damage to myocardium, liver and kidneys, with progressive impairment of renal function and concentrating ability. Spleen changes: in young children the spleen is enlarged, with occasional acute splenic sequestration. Altered splenic function increases the risk of ser ious infection particularly meningitis sepsis caused by pneumococci and haemophilus influenzae (polysaccharide encapsulated organisms). As the child ages, auto splenectomy reduces spleen size. Cardiomegaly is invariably prese nt in older children (sickle-related

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cardiomyopathy), secondary haemosiderosis from increased iron absorption may damage liver, pancreas and heart, and there may be gall stone formation. Puberty is frequently delayed, and chronic leg ulcers occur in late adolescence. 79. Sickle cell trait is usually characterized by all of the following except:A. Red cell concentration of HbS is about 50% B. Splenic infarction rarely occur except under extreme hypoxic condition C. Reticulocyte count is usually elevated D. In some individual hyposthnuria & hematuria may result from sickling in the renal medullary capillaries E. The incidence in American black is 1 in 12 Ans:- C 80. In paroxysmal nocturnal haemoglobinuria:A. The defect is a congenital abnormality of RBC production by the bone marrow. B. Hamoglobinuria is a characteristic finding. C. Venous thrombosis is a recognised complication. D. The diagnostic test is Ham’s test. E. Transforms into acute leukaemia in approx. 5%. Ans:-BCDE PNH is an acquired clonal defect of cell membranes that makes the cells more sensitive to lysis by complement. This results in intravascular haemolysis and haemoglobinuria. Cr ises of haemolysis can be triggered by infection, and chronic haemolysis may result in nephropathy. There is an increased risk of venous thromboses (esp. in unusual sites such as sa ggital sinus or hepatic veins).The diagnostic test is Ham’s test (acidified serum lysis). The disease may transform into acute leukaemia (in approx 5%).

81. The following conditions commonly require splenectomy in later life: A. Sickle cell trait B. Sickle cell disease C. Beta Thalassaemia D. Glucose-6-phosphate dehydrogenase deficiency E. Idiopathic thrombocytopenic purpura true

Ans:- C Comments: Because of the risk of post-operative sepsis, splenectomy should be limited to specific indications. These include: • Splenic rupture, anatomic defects. • Haemolytic anaemias, immune cytopenias. • Metabolic storage diseases. • Secondary hypersplenism. • Surgical indications (rare). The major risk is infection, particularly in children less than 5 years. The risk of sepsis is slightly less in splenectomies done for trauma, red cell membrane defects, and immune cytopenias then when there is a preexisting immune deficiency such as Wiskott-Aldrich Syndrome or ret iculoendothelial blockage such as storage diseases or severe haemolytic anaemias. 82. The following is true of aplastic anaemia:A. Congenital aplastic anaemia is more common than the acquired form B. Fanconi's anaemia is the commonest cause C. Age related anaemia is prerequisite for the diagnosis to be confirmed D. Erythropoietin is an effective treatment in the majority of cases E. Allogeneic bone marrow transplantation is recognized treatment option

Ans:- E 83. Aplastic anaemia:

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A. B. C. D. E.

Is defined as pancytopenia in the peripheral blood and blast cells in the bone marrow. Has been associated with chloamphenicol eye drops. Is preduced by hepatitis in 5-10% of cases Is a feature of paroxysmal nocturnal haemoglobinuria Can effectively be treated by growth factor injections Ans:-CD Aplastic anaemia is defined as the presence of pancytopenia in the peripheral blood and a hypocellular marrow in which normal marrow is replaced by fat cells, abnormal cells are not found in either the peripheral blood or bone marrow. Causes include: drugs, viral infections and paroxysmal nocturnal hemoglobinemia. The latter condition is an acquired defect of the red cell membrane which is associated with intravascular hemolysis as the red cells are highly susceptible to complement lysis. Many drugs have been implicated in aplastic anaemia but the evidence for many is slim. Oral chloramphenicol not eye drops have been associated with aplastic anaemia, hepatitis, presumably of viral origin is a precursor of aplastic anaemia in 5-10% of cases. In the majority of cases no specific hepatitis virus is identified. Definitive treatment is with immunosuppression or with allogenic stem cell transplantation in young patients with HLA-matched doners. Growth factors only have a role acutely to treat sever infection and are not effective as long term treatment 84. These inborn errors have successfully been treated by bone marrow transplantation A. Cystic fibrosis B. Hurler's syndrome. C. Gaucher's syndrome. D. Fanconi's anaemia. E. Chediak-Higashi syndrome.

Ans:-BCDE 85. Regarding leukocytes:A. Eosinophils play role in the defense against parasites B. Mast cell are derived from basophils C. Once migrated into the tissue, neutrophil is called a phagocyte D. Lymphocytes contain myeloperoxidase E. Band cells are immature neutrophils

Ans:-ABE 9

86. 5 year old child is found to have neutrophil count of 51 10 /L this may be due to : A. Acute liver failure. B. Corticosteroid therapy. C. Brucellosis. D. Aplastic anaemia. E. Hypersplenism.

Ans:-ABC 4

Granulocytes survive for only 6–12 hr in the circulation and, therefore, daily production of 2 × 10 granulocytes/μL of blood is required to maintain a level of circula ting granulocytes of 5 × 10 3/μL. In contrast, lymphocytes can exhibit lifetimes measured in months or years and require daily renewal of lymphocyte progenitors at rates substantially lower than the ot her hematopoietic progenitors. The mean WBC count at birth is high, followed by a rapid fall beginning at 12 hr until the end of the 1st week. Thereafter, values are stable until 1 yr of age. A slow, steady decline in the WBC count continues throughout childhood until reaching the adult value during adolescence. Leukopenia in adolescents and adults is defined as a total WBC count <4,000/μL. 87. Neutropenia :A. Is defined as circulatory count < 5´109/L.

ABDULRAHMAN BASHIRE B. C. D. E.


Increase susceptibility to viral infection. Can be caused by corticosteroid. May be cyclical. May be complication of folate deficiency. Ans:-DE

Viral

Respiratory syncytial virus Dengue fever Colorado tick fever Mumps Viral hepatitis Infectious mononucleosis (EBV) Influenza Measles Rubella Roseola Varicella Cytomegalovirus Human immunodeficiency virus Sandfly fever

Bacterial

Pertussis Typhoid fever Paratyphoid fever Tuberculosis (disseminated) Brucellosis Tularemia Gram-negative sepsis Psittacosis

Fungal

Histoplasmosis (disseminated)

Protozoal

Malaria Leishmaniasis (kala-azar)

88. Atypical mononuclear cells are seen with :A. Toxoplasmosis. B. Herpes simplex. C. Rubella D. Cytomegalovirus. E. Epstein-Barr virus.

Ans:-CDE 89. Neutrophil A. Are present in normal tissues. B. Are directed to sites of inflammation by IL18. C. Phagocytes cells more efficiency when opsonised with antibody and complement as compared with antibody alone. D. Kill phagocytosed organisms by releasing granule contents E. Deficiency increases susceptibility to viral infections. Ans :-BCD Neutrophils remain in the blood stream and only enter tissues if there is inflammation (unlike macrophages) to which they are principally directed by IL18. Antibody and complement is a much more efficient opsonin than antibody alone. Neutrophils release toxic oxygen compounds (respiratory burst),nitric oxide, phospholipases and proteases from granules to kill phagocytosed organisms. Deficiency or dysfunction of neutrophils results in recurrent bacter ial and fungal infections 90. The following can cause an eosinophilia:A. Penicillin B. Hydralazine C. Polyarteritis nodosa D. Pneumocystic carini E. Atopic dermatitis.

Ans:- ABCDE

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Blood eosinophil numbers do not always reflect the extent of eosinophil involvement in disease-affect ed tissues. The absolute eosinophil count, calculated as the white blood cell (WBC) count/μL × percent of eosinophils, is usually <450 cells/μL in the blood and varies diurnally, being more abundant in the early morning and diminishing as endogenous glucocorticoid levels r ise. Eosinopenia occurs after corticosteroid administration and with some bacterial and viral infections , The absolute eosinophilia count is used to quantitate eosinophilia. Many diseases are associated with moderately severe (1,500–5,000 cells/μl) or severe (>5,000 cells/μl) eosinophilia, Patients with sustained blood eosinophilia may develop organ damage, especially cardiac damage as found in the idiopathic hypereosinophilic syndrome, and should be monitored for evidence of cardiac disease. Many cases of moderately severe eosinophilia often have no clear et iology. ALLERGIC DISORDERS

INFECTIOUS DISEASES Tissue-Invasive Helminth Infections

MALIGNANT DISORDERS

GASTROINTESTIN AL DISORDERS

RHEUMATOLOGI C DISEASE

Brain tumors

Inflammatory bowel disease

Rheumatoid arthritis

Peritoneal dialysis

Eosinophilic fascitis

I

Trichinosis Toxocariasis Allergic rhinitis

Strongyloidosis Ascariasis Filariasis Schistosomiasis Echinococcosis

Asthma

Pneumocystis carinii

Hodgkin disease and T-cell lymphoma

Acute and chronic urticaria

Toxoplasmosis

Acute myelogenous leukemia

Eosinophilic gastroenteritis

Pemphigoid

Amebiasis

Myeloproliferative disorders

Milk precipitin disease

Malaria

Brain tumors

Chronic active hepatitis

Bronchopulmonary aspergillosis Coccidioidomycosis Scabies

Hodgkin disease and T-cell lymphoma Acute myelogenous leukemia Myeloproliferative disorders

91. These statements are true:A. Methemoglobinemia is caused by oxidation of copper 2 to copper 3 form. B. Methemoglobinemia may be due to HbC, abnormal hemoglobin. C. Methemoglobin reductase maintain Hb in it's reduced state. D. NADH is the energy source used in the redox reaction. E. Production of NADH arise the consequence of conversion of 1, 3 diphosphoglycerate to glyceraldehydes. Ans : -CD

p

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92. The following conditions present with purpura & normal platelet counts:A. Osler-Weber-Rendu syndrome B. Ehler –Danlos syndrome C. Bernard—Soulier syndrome. D. Wiskott-Aldrich syndrome. E. Scurvy.

Ans:-ABE 93. The following are causes of thrombocytopenia: A. B12 deficiency B. Chronic myeloid leukaemia. C. Splenectomy. D. Bernard soulier syndrome E. May-Hegglin anomaly.

Ans :-ABDE B12 deficiency may be associated with a pancytopenia or with an isolated anaemia, thrombocytopenia or leucopenia. Chronic myeloid leukaemia may be associated w ith a low, normal or high platelet count. Bernard soulier syndrome and May Hegglin anomaly are congenital problems of plate let production and are both associated with a thrombocytopenia. Splenectomy is associated with a thrombocytosis as well as increased target cells and Howell Jolly bodies in the peripheral blood. 94. Thrombocytopenia occurs in A pulmonary haemosiderosis. B cavernous haemangioma. C scurvy. D infectious mononucleosis. E chronic alcoholism.

Ans:- BDE Comments: A - blood picture of iron deficiency +/-eosinophilia C - perifollicular haemorrhage due to vascular weakness and impaired platelet function cavernous haemangioma - platelet sequestration chronic alcoholism - suppression of megakaryocytes infectious mononucleosis - immune mediated Thrombocytopenia

II. Signs A. Bleeding disorders B. Purpura or Petechiae III. Causes: Congenital A. Glanzmann's Thrombasthenia (autosomal recessive) 1. Platelet membrane deficiency Glycoprotein IIb, IIIa 2. Defective binding of platelet Fibrinogen 3. Decreased platelet aggregation B. Bernard-Soulier Disease (autosomal recessive) 1. Platelet membrane deficiency Glycoprotein Ib 2. Coagulation Factor X and Factor V deficiency 3. Large platelets and decreased platelet aggregation C. Storage Pool Disease 1. Dense granule and/or alpha granule deficiency 2. Defective platelet release of ADP and Serotonin IV. Causes: Acquired A. Uremia B. Chronic Liver Disease

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C. Medications 1. Aspirin 2. Furosemide (Lasix) 3. Nitrofurantoin (Furadantin ) 4. Heparin 5. Sympathetic blockers 6. Clofibrate (Atromid-S) 7. NSAIDs 95. The following are recognised causes of drug-induced immune thrombocytopenia:A. Chloramphenicol B. Quinine C. Penicillamine D. Rifampicin E. Heparin

Ans:-BDE All the above drugs can cause thrombocytopenia. However chloramphenicol, penicillamine and phenylbutazone cause this by bone marrow suppression. The others produce an antibody-drug-plasma protein complex which deposits on the platelet surface leading to either lysis by complement or removal by reticuloendothelial cells. Patients present with acute purpura and platelets often less than 10x10 9/l. Heparin also causes thrombocytopenia through platelet aggregation. 96. Hemophilia A:A. Is the commonest inherited bleeding disorder. B. Inheritance is autosomal dominant with variable penetrance. C. Severity is determined by level of factor VIIIc D. Is treated with factor VIII given by IM injection. E. Is no longer treated with prophylactic factor VIII because of HIV risk.

Ans:-AC Recombinant factor VIII is not widely available. 97. The following statement concerning hereditary bleeding disorder are correct :A. HCV is common cause of liver disease in adults with sever hemophilia treated before the 1990. B. Antenatal diagnosis of hemophilia A is possible. C. Von-Willebrand disease is inherited as an autosomal recessive manner in the majority of patient. D. Children with hemophilia have normal prothrombin time. E. In hemophilia A , spontaneous bleeding into the joint occur when the factor VIII concentration is reduced to 20% of normal. Ans:-ABD 98. The following condition are caused by an abnormality of Hb synthesis :A. Beta thalassemia B. Congenital spherocytosis C. Pyruvate kinase deficiency D. Hemophilia A E. Sickle cell disease. Ans:-AE 99. Von Willebrand disease (vWD):A. Is caused by pualitative or quantitative deficiency of von Willebrand factor B. The resulting bleeding disorder in vWD is characterized by petechiae C. The platelet count is normal D. The ristocetin co-factor activity will be decreased

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E. Is inherited in an X-linked recessive pattern Ans:-ACD 100.

Hemophilia:A. Doesn't cause coagulation disturbance in the newborn. B. Lack positive family history in 1/3 of the affected boys. C. Cause profound bleeding time. D. Cause prolonged APTT ( test of intrinsic coagulation pathway). E. Result from the synthesis of biologically inactive factor VIII (AHG).

Ans:-BDE 101.

Which of the following is true of Factor VIII antihaemophilic globulin? A. released mainly by megakaryocytes B. mediates the endothelial platelet aggregation C. is an essential co-factor in the activities of Factor X to Factor Xa D. has a half life of 36 hours E. deficiency is a major source of bleeding in Von Willebrand's disease

Ans:-BCDE Comments: factor VIII antihaemophilic globulin / factor = Von Willibrand's factor It is the plasma carrier for factor VIII a-mainly from endothelium but also from megakaryocytes b-it links platelet membrane receptors to vascular subendothelium c-in association with calcium / activated factor IX d-stored blood at 4°C -> activity falls to 10% in first 3 days (Dr Shu Ho) 102.

Regarding coagulation:A. All clotting factor are synthesized in the liver B. Factor VIII is synthesized by hepatocytes and by endothelial ce lls C. Factor CIII deficiency doesn't prolong the INR nor APTT D. An isolated factor VII deficiency elevates the APTT o nly E. Isolated factor II deficiency result in prolonged APTT, thrombin time( TT) and INR Ans:-ABCE Because factor XIII is responsible for the cross linking of fibrin to stabilize the fibrin clot, symptoms of delayed hemorrhage are secondary to instability of the clot. Typically, patients have trauma 1 day and then have a bruise or hematoma the next day. Clinical sympto ms include mild bruising, delayed separation of the umbilical stump beyond 4 wk, poor wound healing, and recurrent spontaneous abortions in women 103.

The following are component of intrinsic coagulation:A. Prothrombine B. Factore CP C. Factore IC. D. Fibrinogen E. Factore VII

Ans:-BC 104.

105.

In neonatal alloimmune thrombocytopenia:A. The most commonly occurring human platelet antigene is HPA-1a B. The father need to be HPA negative and the mother positive for the same HPA for NAIT to develop C. The first born child of genetically predisposed parents is commonly involved. D. The thrombocytopenia become evident after approximetly 5 days of life. E. The treatment includes antenatal periumbilical transfusion of maternal donated platelet. Ans:-ACE Neonatal thrombocytopenia is recognized feature of :A. SLE B. Maternal ingestion of thiazid diuretic.

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C. Maternal IgM antibodies. D. In utero infection with Toxoplasmosis. E. Shortened forearm. Ans ;-ADE 106.

Thrombocytopenia has recognized association with :A. HSP B. Septicemia. C. Rubella. D. Von-Willebrand disease. E. Portal hypertension.

Ans:- BCE 107.

Idiopathic Thrombocytopenic Purpura (ITP) in Childhood: A. Is usually self limiting. B. Always requires treatment if the platelet count is less than 20 x 109/L. C. Is associated with cerebral haemorrhage in less that 1% of cases. D. Can be successfully treated with steroids. E. Can be successfully treated with intravenous immunoglobulins.

Ans:- ACDE Comments: Acute idiopathic thrombocytopenic purpura is the most common of the thrombocytopenic purpuras of childhood. It is associated with petechiae, mucocutaneous bleeding, and occasionally haemorrhage into the tissues. There is a profound deficiency of circulating platelets despite adequate numbers of megakaryocytes in the marrow. In 70% of cases there is an antecedent disease or viral infect ion. ITP has an excellent prognosis even when no specific therapy is given. Within 3 months, 75% of patients recover completely, mostly within 2 months. Spontaneous haemorrhage and intracranial bleeding (<1%) are usually confined to the initial phase of disease. Thereafter, spontaneous manifestations subside. 90% of children have a normal platelet count within a year, and re lapses are unusual. FFP or platelet concentrates have transient benefit only, but should be administered when life t hreatening haemorrhage occurs. When the disease is mild and haemorrhages of the retina or mucus membranes are not present, no specific therapy is given. Gammaglobulin and corticosteroid may be used in severe cases. The for mer produces a more rapid effect, and the latter may disguise the occasional case of leukaemia presenting with thrombocytopenia. 108.

In ITP:A. The purpuric rash is characteristically on the buttock and the extensor surface of the limbs. B. Bone marrow examination characteristically reveals diminished numbers of megakaryocytes. C. The infant of an affected mother may develop transient thrombocytopenia. D. Steroid therapy is ineffective E. The prothrombin time is prolonged Ans :-C 109. Concerning ITP in childhood A. Bed rest is indicated B. Bone marrow examination is essential C. Incidence is 1 in 1000 children per year D. Platelet transfusion is necessary if the platelet count fall below 20 E. I/V immunoglobulin may be beneficial Ans:-E Antiplatelet antibodies bind to transfused platelets as well as they do to autologous platelets. Thus, platelet transfusion in ITP is usually contraindicated unless life-threatening bleeding is present. (safe level of >20 × 109/L,) 110.

Idiopathic thrombocytopenia purpura: A. Result from decreased platelet production. B. Classically present with acute blood loss.

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C. Require urgent treatment to prevent intracranial hemorrhage. D. Should be confirmed by BM aspirate if there is no response to steroid therapy. E. Is preceded by viral URTI in more than 60% of cases. Ans:-E 111.

ITP:A. B. C. D. E.

Carries 1% risk of intracranial hemorrhage if the platelet count fall below 20´109/L Should be treated by Splenectomy if the platelet count is<10´109/L. Is due to impaired platelet production by the bone marrow. Bone marrow examination is essential if immunoglobulin treatment is to be used. Resolve spontaneously within 6 months in the majority of childhood cases Ans:-AE

Splenectomy in ITP should be reserved for:-. The older child (> 4 yr) with severe ITP that has lasted >1 yr (chronic ITP) . Whose symptoms are not easily controlled with therapy is a candidate for splenectomy. Splenectomy must also be considered when life-threatening hemorrhage (intracranial hemorrhage) complicates acute ITP, if the plate let count cannot be corrected rapidly with transfusion of platelets and administration of IVIG and corticosteroids. Splenectomy is associated with a lifelong risk of overwhelming postsplenectomy infection caused by encapsulated organisms. 112.

Disseminated intravascular coagulation: A. Is usually associated with a high fibrinogen level. B. Is usually associated with a normal thrombin time. C. May be associated with cytomegalovirus or varicella zoster infections. D. May occur in liver disease. E. May be associated with formation of microthrombi, so should be treated with intravenous heparin Ans:-CD * Disseminated intravascular coagulation (DIC) may be caused by bacterial infections, in particular gra mnegative and meningococcal septicemia, by severe falciparum malar ia infections and by viral infections including CMV, HIV and varicella zoster. * Liver disease can also cause DI C due to release of thromboplastins from damaged liver cells a nd reduced concentrations of antithrombin and protein C. * Investigations show a prolonged thrombin time, prothrombin time and activated partial thromboplastin time (aptt), thrombocytopenia, a low fibrinogen and raised D-dimers or FDPs. * DIC may be associated with microthrombin causing skin lesions, r enal failure, gangrene or cerebral ischemia but heparin therapy is usually not indicated as it can aggravate the bleeding tendency. 113.

Feature of Wiskott-Aldrich syndrome include: A. Autosomal recessive inheritance B. Normal platelet count C. T cell defect D. Death from acute hemorrhage in 20% E. Eczema

Ans:-CDE Prenatal diagnosis and carrier dete ction is possible in 98%, Low serum IgM , normal IgG and raised IgA &IgE, Examination of the bone marrow in WAS shows the normal number of megakaryocytes, although they may have bizarre morphologic features. Transfused platelets have a normal life span. Splenectomy often corrects the thrombocytopenia, suggesting that the platelets formed in WAS have acce lerated destruction. Approximately 5% of patients with WAS develop lymphoreticular malignancies. Successful bone marrow transplantation cures WAS.

ABDULRAHMAN BASHIRE 114.


Neonatal thrombocytopenia is recognized feature of:A. SLE B. Maternal ingestion of thiazide diuretics C. Maternal IgM antibodies D. In utero infection with toxoplasmosis E. Shortened forearm.

Ans :-ADE Neonatal thrombocytopenia often occurs in association with congenital viral infection, especially rubella and cytomegalovirus; protozoal infection, such as toxoplasmosis; syphilis; and bacterial infection, especially those caused by gram-negative bacilli, thrombocytopenia and abnormal abdominal findings is common in necrotizing enterocolitis. 115.

Neonatal thrombocytopenia:A. Is an indication for cerebral ultrasound B. Is an absolute indication for platelet transfusion after delivery. C. May be caused by toxoplasmosis infection during pregnancy D. Can occur in infant born to mother with SLE E. May be associated with absent radii

Ans:-ACDE Neonatal isoimmune thrombocytopenia occurs in neonates who are platelet antigen (PLA1) positive born to PLA1 negative mothers, sensitization occur in utero & first pregnancy can be affected. 2% of population is PLA1 negative, 89% of population is PLA1 positive, but only 6% of PLA1 negative mothers with positive fetuses will develop antibodies. 10% of affected fetuses will have intraventricular hemorrhage with high associated morbidity and mortality. Monitoring of maternal antibodies can be performed during pregnancy and platelet transfusion of PLA1 negative platelets given to infants at risk. 116.

Glanzmann's thrombasthenia:A. Is inherited as an autosomal dominant B. Gene locus is known C. Platelet count is normal D. Require long term steroid treatment E. Splenectomy may be beneficial

Ans:-BC 117.

In which of the following organ is Erythropoietin primarily produced:A. Bone marrow B. Liver C. Kidney D. Spleen E. Intestine

Ans:- C 118.

Regarding the red cell: A. Carbon Dioxide binds with reduced haemoglobin. B. The oxygen affinity of haemoglobin is decreased in the presence of acidosis. C. The oxygen affinity of fetal haemoglobin is greater than adult haemoglobin. D. Carbonic anhydrase is present in all red cells. E. Most carbon dioxide in venous blood is transported bound to albumin.

Ans:-ABCD Comments: Carbon dioxide is carried in the blood in 3 forms: • Dissolved (10%). • Bicarbonate, whose formation is encouraged by carbonic anhydrase present in the red cell. • As carboamino compounds: hydrogen ions liberated from the bicarbonate

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reaction are bound to haemoglobin which encourages the release of oxygen, since reduced haemoglobin is less acid than the oxygenated forms. Thus, the presence of reduced haemoglobin in the peripheral blood helps with the loading of carbon dioxide, while the oxygenation which occurs in the pulmonary capillary assists in the unloading of it. The fact that the deoxygenation of the blood increases its ability to carry carbon dioxide is often known as the Haldane effect. Fetal haemoglobin contains g polypeptide chains in place of t he b-chains of Hb A. Its resistance to denaturation by strong alkali is used in its quantitations. Hb F is the predominant haemoglobin from 8 weeks gestation, and constitutes 90% of the total haemoglobin of the 6 month fetu s. At birth 70% of the total is Hb F, and synthesis decreases rapidly postnatally, such that by a year, only 2% is present. Hb F has a gr eater oxygen affinity than Hb A, so the growing fetus is preferentially ourished by oxygen in utero. 119.

Hemoglobin A. At 40 week gestation is 70-75% HbF B. At 40 week gestation is 20-25% HbA C. After age 3 is over 98% HbA D. After age 3 year is approximately 3% HbF E. In beta thalassemia minor contain elevated HbF & HbA2

Ans :-ACE Two hemoglobin gene clusters are involved in the production of hemoglobin and are located at the end of the short arm of chromosomes 16 and 11, respectively. On chromosome 16, there are 3 genes within the alpha (α) gene cluster: zeta (ζ) and 2 α genes (α1, α2). On chromosome 11, there are 5 genes within the beta (β) gene cluster: epsilon (ε), delta (δ), beta (β), and 2 gamma (γ) genes. The order of gene expression within each cluster roughly follows the order of expression during the embryonic period, the feta l period, and eventually childhood. 8 wk of fetal life, the embryonic hemoglobin are formed: Gower-1 (ζ2ε2), Gower-2 (α2ε2), and Portland (ζ2γ2). In embryos of 4–8 wk gestation, the Gower hemoglobins predominate, but by the 3rd month they have disappeared. At 9 wk of fetal life , the major hemoglobin (Hb) is Hb F (α2γ2). Its resistance to denaturation by strong alkali is the basis for determining the presence of fetal RBCs in the maternal circulation (the KleihauerBetke test ). After the 8th gestational wk, Hb F is the predominant hemoglobin; at 24 wk gestation it constitutes 90% of the total hemoglobin. During the 3rd trimester, a gradual decline occurs, so that at birth Hb F averages 70% of the total. Synthesis of Hb F decreases rapidly postnatally, and by 6–12 mo of age only a trace is present. At approximately 1 mo of fetal life , Hb A (α2β2) appears, but does not become the dominant hemoglobin until after birth, when Hb F levels start to decline. A minor hemoglobin, Hb A 2(α2δ2) appears shortly before birth and remains at a low level after birth. The final hemoglobin distribution pattern that occurs in childhood is not achieved until at least 6 mo of age, and sometimes later. The normal hemoglobin pattern is >95% Hb A, ≤3.5 Hb A2, and <2.5% Hb F. Some Hb A (α2β2) can be detected in even the smallest embryos. Accordingly, it is possible as early as 16– 20 wk gestation to make a prenatal diagnosis of ma jor β-chain hemoglobinopathies, such as thalassemia major . Prenatal diagnosis is based on techniques that examine the rat es of synthesis of β chains or the structure of newly synthesized β chains. Earlier diagnosis is possible using molecular biology techniques and sampling of chorionic villus tissue or amniotic fluid if DNA structural defects are a cause of the hemoglobinopathies. Gene deletion disorders such as the α- thalassemias can be detected using the same method. By the 24th wk of gestation, 5–10% of Hb A is present. A steady increase follows, so that at term, Hb A averages 30%. By 6–12 mo of age, the normal Hb A pattern appears. The minor Hb A component Hb A2contains delta (δ) chains and has the structure α2δ2. It is seen only when significant amounts of Hb A are also present. At birth, <1.0% of Hb A2is seen, but by 12 mo of age the normal level of 2.0–3.4% is att ained. Throughout life, the normal ratio of Hb A to A 2is about 30:1.

ABDULRAHMAN BASHIRE 120.


hemoglobin; th A. Is present in blood cells by the 14 day after gestation. B. Is predominantly produced in the liver at term. C. At term is mainly fetal hemoglobin. D. Production increase after delivery. E. Concentration at birth is 10-12 g/dl

Ans:- AC Mesoblastic hematopoiesis occurs in extraembryonic structures, principally in the yolk sac, and begins between the 10th and 14th days of gestation. By 6–8 wk of gestation the liver replaces the yolk sac as the primary site of blood cell production, and by 10–12 wk extraembryonic hematopoiesis has essentially ceased. Hepatic hematopoiesis occurs in the liver throughout the remainder of gestation, although production begins to diminish during the second trimester as bone marrow (myeloid) hematopoiesis increases. The liver remains the predominant hematopoietic organ through wk 20–24 of gestation 121.

The O2-Hb dissociation curve is displaced to the right in: A. Fetal Hb B. Methemoglobinemia C. Increased lactic acid production D. Chronic hypoxia due to cyanotic conditions E. Increased 2,3 DPG

Ans:-CDE When shifted to the right, O2 is easily given up and this will be bene ficial in conditions when the body is working hard. * Increase in CO2 production due to increased metabolism * Increase in H+ * Hyperthermia * Chronic hypoxic states/anemia, where there is an increase in 2,3 DPG 122.

en uptake by haemoglobin is characterized by: A. Alkalosis. B. Raised temperature. C. Increased 2-3 DPG. D. Increased red cell volume and MCH. E. High altitude.

Ans:-BCE 99% of the oxygen which dissolves in blood is transported by the oxygen carrying compound haemoglobin. Haemoglbin is composed of four globin subunits (in normal adults – two alpha and two beta globin chains) each of which contains a haem group. Haem is a c omplex of prophyrin and ferrous iron. The oxygenhaemoglobin dissociation curve relates the saturation of haem with oxygen as a % (the y axis) to t he PO2 in mmHg(on the x axis). This curve is affecte d by a number of factors: 1- PH – a fall in pH shifts the curve to the right i.e a higher PO2 is required for haemoglobin to bind oxygen. This is known as the Bohr effect. An increase in CO2 (as is found in peripheral tissues) will cause a fall in pH, and the associated decrease in affinity of haemoglobin for oxygen contributes to the transfer of oxygen from the blood in to the tissues. Alkalosis will have the opposite effect. 2- Temperature – A rise in temperature shifts the curve to the right i.e oxygen uptake by haemoglobin is decreased. 3- 2,3-DPG levels- this substance is formed by glycolosis via the Embden-Meyerhof pathway. It binds to the beta-globin chains of deoxyhaemoglobin and causes oxygen to dissociate from Hb. A rise in 2,3-DPG shifts the oxygen dissociation curve to the right releasing more O2. 4- High altitude results in a rise in 2,3-DPG concentrations in RBCs, with an increase in the availability of oxygen to the tissues. The greater affinity of foetal haemoglobin for oxygen as compared to its adult counterpart is a result of the decreased binding of 2,3-DPG by the foetal gamma globin chain.

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5- With regards RBC indices, it is the MCHC which affects the oxygen-dissociation curve, rather than the MCH or MCV. In anaemia, the MCHC is reduced, and the curve is right shifted, increasing the release of oxygen to the tissues. 123.

Hazard of blood transfusion include :A. Haemosiderosis. B. Circulatory failure. C. Hypokalemia. D. DIC E. Cytomegalovirus infection.

Ans:-ABDE 124.

Red cell physiology A. Erythropoietin is produced predominantly by endothelial cells, in response to reduction of partial pressure of oxygen in the blood stream B. Vitamin C is coenzyme required in the initial stage of haem synthesis . C. The mitochondria & ribosomes within the erythroblast produce haem & globin respectively D. Molecule of hemoglobin is comprised of four globin chains attached to their own haem moiety E. The red cell generates its own energy by metabolizing glucose only Ans:-CDE 125. Arterial thromboses are associated with:A. Behcets syndrome B. Atrial fibrillation C. Factor V Leiden mutation D. Protein C deficiency E. Polycythaemia Ans:-ABE Arterial thromboses may be associated with Behcets syndrome, atrial fibrillation and polycythaemia. Factor V Leiden mutation and protein C deficiency are assoc iated with venous thromboses 126.

Which of the following is not a recognised complication of anticoagulation therapy? A. Deep venous thrombosis. B. Thrombocytopaenia. C. Skin necrosis. D. Osteoporosis. E. Peripheral neuropathy.

Ans:-E Although the main side effect of anticoagulation therapy is excessive bleeding, more unusual side effect exist. Heparin may be associated with both thrombocytopaenia and thrombosis as part of t he disorder known as heparin-indused thrombocytopaenia (HITT). This is an acquired immune disorder in which heparindependant anti-platelet antibodies form, leading to sever thrombocytopaenia and occasionally catastrophic thrombosis. Treatment involves stopping heparin and anticoagu lating with hirudin or danaparoids. Longterm heparin therapy may be associated with osteoporosis. Warfarin, especially induction therapy in patients with inherited protein C or S deficiency, may be associated with skin necrosis. 127.

Low A. B. C. D. E.

protein C levels are not seen with: Warfarin therapy Neonatal purpura fulminans Factor V Leiden Chicken pox Meningococcal septicaemia

Ans:- C

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Protein C is a vitamin K-dependent protein synthesised in the liver. It circulates in an inactive form, which becomes activated by thrombin/thrombomodulin complexes on the surface of endothelial cells. Activated protein C inhibits coagulation by degrading coagulation factors Va and VIIIa and resulting in decreased thrombin formation. It requires protein S as a cofactor. Causes of protein C deficiency are: 1) Congenital * homozygous deficiency - those with severe deficiency - (protein C levels <1% of normal) present with severe neonatal purpura fulminans, cerebral thrombosis and DIC. Those with milder defects have prote in C levels of 10 -24 % of normal. They can present w ith massive venous thrombosis as older children. * heterozygous deficiency (protein C levels 30-40% of normal) most frequently presents a fter puberty with deep venous thrombosis of the lower limb. It can also present w ith recurrent superficial thrombophlebitis and DVT. Most thrombotic episodes occur spontaneously but known associated risk factors include surgery with immobilisation, pregnancy and the oral contraceptive. Many people remain asymptomatic. A positive family histor y of thrombosis is associated with an increased risk of symptoms - + - 50% of individuals with heterozygous protein C deficiency and a family history of thrombosis, will experience a thrombotic event. 2) Causes of acquired protein C deficiency: * infections - meningococcal disease and varicella infection * DIC * hepatic disease * drugs - warfarin, chemotherapeutic agents e.g. cyclophosphamide, methotrexate, 5-fluorouracil), lasparaginase * malignancy - acute myeloid leukaemia * chronic inflammation - inflammatory bowel disease * haemolytic uraemic syndrome * thrombotic thrombocytopaenic purpura Patients with protein C deficiency are at high risk for warfarin-induced skin necrosis during initiation of therapy with warfarin. Factor V Leiden refers to a point mutation in the gene for factor V results in resistance of factor V to the actions of activated protein C, and consequently a thro mbophillic state. Protein C levels are not decreased QUESTION NUMBER 4637 Patients with iron overload may have endocrine dysfunction. Endocrine dysfunction in patients with iron overload is likely to spare which of the following organ functions: a) Pituitary function b) islet cell function c) thyroid function d) ACTH production e) parathyroid activity Correct answer: C EXPLANATION Dysfunction of the endocrine pancreas is common in patients with iron overload. Some people develop overt diabetes mellitus requiring insulin therapy. The disturbances in carbohydrate metabolism are often mor e subtle, however. An oral glucose tolerance test o ften unmasks abnormal insulin production. Vigorous exorcism of the excess iron occasionally reverses the islet cell dysfunction (Bomford and Williams, 1976). Exocrine pancreatic function, in contrast, is usually well-preserved. Pituitary dysfunction produces a plethora of endocrine disturbances (Costin et al., 1979). Reduced gonadotropin levels are common. When coupled with primary reductions in gonadal synthesis of sex steroids, this phenomenon delays sexual maturation in some children with transfusional iron overload. Secondary infertility is common (Schafer et al., 1981). Although Addison's syndrome is uncommon with iron overload, production of ACTH is occasionally deficient. A metapyrone stimulation test shows delayed or diminished pituitary secretion of ACTH (Schafer et al., 1985). Thyroid function is usually well-preserved in patients with iron overload. In contrast, parathyroid activity is

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frequently compromised. Functional hypoparathyroidism can be demonstrated in many patients by inducting hypocalcemia with an intravenous bolus of ethelyenediamine tetr aacetic acid (EDTA) while monitoring the production of parathyroid hormone QUESTION NUMBER 4547 The most typical finding of severe folate deficiency in a blood smear : a) fragmentation of the cytoplasm of megakaryocytes b) lobulated macrocytosis c) Hypersegmentation of neutrophils d) megaloblastic erythroblasts e) giant metamyelocytes

Correct answer: C EXPLANATION The blood film is likely to show oval macrocytes and hypersegmented neutrophil nuclei (with six lobes). In severe cases, the white cell count and platelet count also fall (pancytopenia). The bone marrow shows characteristic megaloblastic erythroblasts and giant metamyelocytes (early granulocyte precursors). Biochemically, there is a increase in plasma of unconjugated bilirubin and serum lact ic dehydrogenase, with, in severe cases, an absenc of haptoglobins and presence in urine of haemosiderin. The features of both B12 and folate deficiency include an E levated MCV and low reticulocyte count. However, many have normal indices because of coexistent t halassemia or iron deficiency, etc. RBC folate level is preferred because serum folate level varies with meals and is an unreliable indicator of base state. Thrombocytopenia (50%) and leukopenia are late findings. Smear shows anisocytosis, poikilocytosis, basophilic stippling, and hypersegmentation of neutrophils. Once the diagnosis of vitamin B12 deficiency is made, a Schilling test can identify the etiology, although it is optional. QUESTION NUMBER 4816 a water soluble ferric heme containing enzyme that reduces hydrogen peroxide to water. a) malate dehydrogenase b) Cytochrome c peroxidase c) 5-alpha reductase d) protein C e) pepsin

Correct answer: B EXPLANATION water soluble ferric heme containing enzyme in the peroxidase family of enzymes that t akes reducing equivalents from cytochrome c and reduces hydrogen peroxide to water. QUESTION NUMBER 942 A 13 year old female has swollen cervical lymph nodes and is febrile. Her blood culture is positive for staphylococcus aureus. She shows other signs of being septic. The intern attending her t ells her associate that the patient's WBC count is 15,000 cc/mm with a shift to the left. What does she mean by a 'shift to t he left?' a) increased number of white blood cells in the peripheral blood b) decreased number of white blood cells in the peripheral blood c) hypersegmented granulocytes present in the peripheral blood d) increased number of immature white blood cells in the peripheral blood e) increased number of immature red blood cells in the per ipheral blood Correct answer: D EXPLANATION A shift to the left refers to an increase number of immature white blood cells in the peripheral smear. Typically, the immature white blood cells are being r eleased prematurely by the bone marrow in order to combat some sort of infection.A increased number of white blood cells in the peripheral blood ( choice a) is

Mcq of Hematology

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