Step 1 Express 2018-Hematology and Oncology

First Aid Express 2018 workbook: HEMATOLOGY & ONCOLOGY

page 1

Hema matol tology ogy & Onco ncolog logy y Questions ANATOMY AN ATOMY 1.

Define the following terms. (p 396) A.

Anisocytosis _______________________________________________________________

B.

Poikilocytosis ______________________________________________________________

C.

Reticulocyte _______________________________________________________________

2.

What do the dense granules of platelets contain? (p 396) ________________________________

3.

What do the α-granules of platelets contain? (p 396) ____________________________________

4.

List the types of white blood cells in order of decreasing prevalence. (p 396) _________________ ______________________________________________________________________________

5.

What conditions can cause hypersegmentation of neutrophils? (p 396) ______________________ ______________________________________________________________________________

6.

CD14 is a cell surface marker for which cell type? (p 397) ________________________________

7.

What seven conditions can cause eosinophilia? (p 397) __________________________________ ______________________________________________________________________________

8.

What molecules are released by basophils? (p 397) ____________________________________ ______________________________________________________________________________

9.

B lymphocytes are produced produced in the _______________ _______________ (bone (bone marrow/thymus) marrow/thymus) and mature in the _______________ (bone marrow/thymus). marrow/thymus). T lymphocytes are produced in the _______________ (bone marrow/thymus) and mature in the _______________ (bone marrow/thymus). (p 398)

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page 2 10.


What type of cell has an off-center nucleus, abundant RER, and a clock-face chromatin distribution? (p 399) _________________________________________________________________________

PHYSIOLOGY 11.

Why are Rh-negative mothers given anti-D IgG? (p 400) __________________________________ ______________________________________________________________________________

12.

In the chart, check which coagulation factors are in the intrinsic vs. the extrinsic coagulation pathways. (p 401) Factor

Extrinsi c Pathway

Intrinsi c Pathw ay

Both Pathw ays

I II V VII VIII IX X XI XII

13.

Which factors are vitamin K dependent? How does warfarin inhibit the activation of these factors? (p 402) _________________________________________________________________________

______________________________________________________________________________


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14.

Describe the four steps of primary hemostasis and platelet plug formation. (p 403) ____________ ______________________________________________________________________________ ______________________________________________________________________________

PATHOLOGY 15.

Identify each cell type and its associated pathology. (pp 404-405)

A.

16.

B.

C.

D.

E.

F.

A. _______________________________

D. ______________________________

B. _______________________________

E. ______________________________

C. _______________________________

F. ______________________________

In α-thalassemia, what is the condition called when all four α-globin genes are deleted? When three are deleted? When two are deleted? W hen one is deleted? (p 406) __________________________ ______________________________________________________________________________

17.

What clinical manifestations do folate deficiency and vitamin B12 deficiency have in common? What sets them apart? (p 408) __________________________________________________________ ______________________________________________________________________________

18.

What are four causes of aplastic anemia? (p 409) ______________________________________ ______________________________________________________________________________


page 4 19.

20.


Match the intrinsic hemolytic normocytic anemia with its characteristic. (p 410) _____ A. G6PD deficiency

1.

GLU  LYS mutation

_____ B. HbC defect

2.

GLU  VAL mutation

_____ C. Hereditary spherocytosis

3.

Heinz bodies

_____ D. Paroxysmal nocturnal hemoglobinuria

4.

Increased incidence of acute leukemias

_____ E. Pyruvate kinase deficiency

5.

Increased fragility in osmotic fragility test

_____ F. Sickle cell anemia

6.

Rigid RBCs

Which autoimmune hemolytic anemias are warm and which are cold? (p 411) ________________ ______________________________________________________________________________

21.

Indicate whether the lab findings in the chart are elevated, decreased, or normal. (p 412) An emi a of Lab Value

Chronic Disease

Iron Deficiency

Pregnancy/ OCPs

Hemochromatosis

Ferritin Serum iron Transferrin % Transferrin

22.

In lead poisoning what enzymes are affected and what substrates are accumulated? (p 413) ____ ______________________________________________________________________________

23.

What are the “5 P’s” of acute intermittent porphyria? (p 413) _____________________________ ______________________________________________________________________________ ______________________________________________________________________________


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24.

Indicate whether the lab findings in the chart are elevated, decreased, or normal. (pp 414-416)

Disorder

Platelet

Bleeding

Count

Time

PT

PTT

DIC Glanzmann thrombasthenia Hemophilia TTP Vitamin K deficiency von Willebrand disease

25.

Which type of Hodgkin lymphoma is most common? (p 417) ______________________________ ______________________________________________________________________________

26.

What type of cell is shown by the arrow in the image? In what condition is this cell seen? (p 417) ________________________________________ ________________________________________ ________________________________________

27.

Match the disease with the genetic translocation most closely associated with it. (pp 418, 420) _____ A. Acute myelogenous leukemia

1.

t(8;14)

_____ B. Burkitt lymphoma

2.

t(9;22)

_____ C. Chronic myelogenous leukemia

3.

t(11;14)

_____ D. Follicular lymphoma

4.

t(14;18)

_____ E. Mantle cell lymphoma

5.

t(15;17)


page 6 28.


What will serum protein electrophoresis reveal in a patient with multiple myeloma? What will be seen in the urine? (p 419) _________________________________________________________

29.

What are the four major groups of leukemia? Which type is at risk for DIC upon initiation of treatment and why? (p 420) ___ _____________________________________________________________ _______________________________________________________________________________ _______________________________________________________________________________

30.

Indicate whether the lab findings in the chart are elevated, decreased, or normal. (p 421) Chronic Myeloproliferative

Platelets

RBCs

WBCs

Disorder

CML Essential thrombocytosis Myelofibrosis Polycythemia vera

PHARMACOLOGY 31.

What is the mechanism of action of heparin? How is overdose treated? (p 423) ______________ ______________________________________________________________________________

32.

What is the mechanism of action of warfarin? How is overdose treated? (p 424) _____________ ______________________________________________________________________________


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33.

Match the drug with its target. (pp 425-431) _____ A. Abciximab

1. ADP receptor (platelets)

_____ B. Clopidogrel

2. B cells (CD20)

_____ C. Etoposide

3. bcr-abl tyrosine kinase

_____ D. 5-Fluorouracil

4. Estrogen receptor

_____ E. Imatinib

5. Glycoprotein IIb/IIIa receptor

_____ F. Rituximab

6. HER-2 (c-erbB2)

_____ G. Tamoxifen

7. Plasminogen

_____ H. tPA

8. Thymidylate synthase

_____ I. Trastuzumab

9. Topoisomerase II

_____ J. Vincristine 34.

10. Tubulin

Match the patient with the drug he or she is most likely taking. (pp 427-428) _____ A. Patient with previous bone marrow transplantation has PFTs consistent with restrictive lung disease _____ B. Patient with colon cancer has myelosuppression not reversible with leucovorin _____ C. Patient with leukemia has myelosuppression reversible with leucovorin

1. Bleomycin 2. Busulfan 3. Cyclophosphamide 4. Doxorubicin 5. 5-Fluorouracil 6. Methotrexate

_____ D. Patient with non-Hodgkin lymphoma has hemorrhagic cystitis _____ E. Patient with solid tumor has dilated cardiomyopathy _____ F. Patient with testicular cancer has PFTs consistent with restrictive lung disease


page 8


An swers ANATOMY 1.

A.

Anisocytosis: Cells vary in size.

B.

Poikilocytosis: Cells vary in shape.

C.

Reticulocyte: Immature RBC.

2.

ADP and calcium.

3.

von Willebrand factor, fibrinogen, and fibronectin.

4.

Neutrophils, lymphocytes, monocytes, eosinophils, and basophils.

5.

Vitamin B12 and folate deficiency.

6.

Macrophages.

7.

Parasites, asthma, Churg-Strauss syndrome, chronic adrenal insufficiency, myeloproliferative disorders, allergic processes, and neoplasia (eg, Hodgk in lymphoma).

8.

Histamine, heparin, and leukotrienes.

9.

Bone marrow; bone marrow; bone marrow; thymus.

10.

Plasma cell.

PHYSIOLOGY 11.

To prevent the development of anti-Rh IgG, which can cause erythroblastosis fetalis in a subsequent fetus.


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12. Factor

Extrinsi c Pathway

Intrinsi c Pathw ay

Both Pathw ays

I

√

II

√

V

√

VII

√

VIII

√

IX

√

X

13.

XI

√

XII

√

Factors II, VII, IX, X, and proteins C and S. Warfarin inhibits the enzyme vitamin K epoxide reductase, which is necessary to convert vitamin K to its reduced form so that it can go on to activate these factors.

14.

1. Injury: Endothelial damage occurs. 2. Exposure: Prompts vWF binding to exposed collagen. 3. Adhesion : Platelets bind vWF via the GpIb receptor and release ADP/Ca2+. 4. Activation : ADP binds the ADP receptor, and GpIIb/IIIa is expressed. Agg reg ati on : Fibrinogen

binds GpIIb/IIIa and links platelets.


page 10


PATHOLOGY 15.

A = Degmacyte (bite cell): G6PD deficiency. B = Ringed sideroblast: sideroblastic anemia. C = Schistocyte: microangiopathic hemolytic anemia (DIC, TTP, HUS). D = Dacrocyte (teardrop cell): bone marrow infiltration (eg myelofibrosis). E = Target cell: HbC disease, asplenia, liver disease, thalassemia. F = Heinz bodies: G6PD deficiency.

16.

Four deletions: Hb Barts; three deletions: HbH disease; two deletions: α-thalassemia minor; one deletion: α-thalassemia minima.17.Both folic

acid

and

vitamin

B12 deficiency

can

cause

megaloblastic anemia. Only vitamin B12 deficiency, however, is associated with neurologic symptoms, such as subacute combined degeneration. Also B12 deficiency is associated with increased methylmalonic acid, unlike folic acid deficiency. 18.

Idiopathic, radiation and drug exposure, viral agents, and Fanconi anemia.

19.

A-3, B-1, C-5, D-4, E-6, F-2.

20.

Warm

(IgG):

SLE,

chronic

lymphocytic

leukemia,

and

certain

drug

reactions.

Cold (IgM): chronic lymphocytic leukemia, Mycoplasma pneumoniae infection, and infectious

mononucleosis. 21. An emi a of Lab Value

Chronic Disease

Deficiency

Hemochoromatosis

OCPs

Ferritin

↑

↓

normal

↑

Serum iron

↓

↓

normal

↑

Transferrin

↓

↑

↑

↓

normal

↓↓

↓

↑↑

% Transferrin

22.

Pregnancy/

Iron

Enzymes: Ferrochelatase and ALA dehydratase. Substrates: Protoporphyrin and ALA (blood)


page 11


23.

Painful abdomen, Port wine-colored urine, Polyneuropathy, Psychological disturbances, and Precipitated by drugs (eg, cytochrome P-450 inducers), alcohol, and s tarvation.

24.

Disorder

Platelet

Bleeding

Count

Time

PT

PTT

↓

↑

↑

↑

Glanzmann thromboasthenia

normal

↑

normal

normal

Hemophilia

normal

normal

normal

↑

↓

↑

normal

normal

Vitamin K deficiency

normal

normal

↑

↑

von Willebrand disease

normal

↑

normal

normal / ↑

DIC

TTP

25.

Nodular sclerosing.

26.

Reed-Sternberg cell; Hodgkin lymphoma.

27.

A-5, B-1, C-2, D-4, E-3.

28.

An M-spike, representing a monoclonal antibody; Ig light chains (Bence Jones proteins).

29.

AML, ALL, CML, and CLL. AML can be associated with DIC upon initiation of treatment because the leukemia cells contain Auer rods composed of the enzyme peroxidase, which is released into the bloodstream when treatment causes cells to lyse.


page 12


30. Chronic Myeloproliferative

Platelets

RBCs

WBCs

Disorder

CML Essential thrombocytosis Myelofibrosis Polycythemia vera

↑

↓

↑

↑

—

—

variable

↓

variable

↑

↑

↑

PHARMACOLOGY 31.

Heparin activates antithrombin, decreasing thrombin and Xa. Treat heparin overdose with protamine sulfate.

32.

Warfarin interferes with normal synthesis and γ-carboxylation of vitamin K-dependent clotting f actors. Treat warfarin overdose with vitamin K, fresh frozen plasma, or PCC.

33.

A-5, B-1, C-9, D-8, E-3, F-2, G-4, H-7, I-6, J-10.

34.

A-2, B-5, C-6, D-3, E-4, F-1.


Step 1 Express 2018-Hematology and Oncology

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