Hematomato board review
By KT/V
CLL • Absolute lymphocyte > 5000 • Monoclonal B cell: CD5, CD 19, 20, CD 23
Blood smear • Smudge cells • Prolymphocyte <10% – If prolymph>55%... PLL
• • • •
Anemia, thrombocytopenia AIHA ITP PRCA
Prognostic factors • • • • •
Ig variable region heavy chain genen mutation ZAP 70 Cytogenetics CD 38 Lymphocyte doubling time
• LDH…. Normal • May transform to aggressive large B cell/ HL – Richter’s transformation
When to treat? • • • • • •
Constitutional symptoms due to CLL Symptom or >10 cm lymphadenopathy Symptom >6 cm below LCM, splenomegaly\ Progressive marrow failure Lymphocyte doubling time <6 mo Autoimmune cytopenia poorly response to steroid
How to treat? • Alkylating- COP • Fludarabine • Rituximab – First infusion: systemic cytokine release syndrome – Hypotension, bronchospasm
• Alemtuzumab – anti CD 52 for fludarabine refractory CLL
CMMoL • Age 70 years male • Myelodysplastic • Fatigue, wt loss, night sweats CRITERIA DX • Monocyte>1000, > 3mo • Absence of philadelphia chromosome • Blast <20% • Dysplasia • Clonal cytogenetic – Trisomy 8, deletion 7q – Translocations 5q31-35; PDGFR, eosinophilia
Treatment • • • • •
All experimental Cytopenia– growth factors Preleukemic– low dose CMT Proliferative– hydroxyurea PDGFR translocation– Imatinib
NHL • Ann arbor staging
Indolent • • • • •
Grade I, II follicular lymphoma B-CLL/SLL Marginal zone lymphoma Lymphoplasmacytoid lymphoma Mycosis fungoides
Treatment- indolent • CLL--- Fludarabine, Rituximab • LPL/Waldenstrom – Fludarabine, rituximab – COP
• Marginal zone – Splenic marginal zone– splnectomy, tx HCV – Asso. H. pylori– eradicate
• Mycosis fungoides– cutaneous T cell – Topical treatment, oral isptretinoin – Sezary syndrome: late occur of nodal and leukemic dz, poor prog
Aggressive • Mantle cell: Male, 70 yr • DLBCL: CD19, 20 • Primary effusion lymphoma: HHV 8 • Treatment R-CHOP: Rituximab, Cyclophos, Doxorubicin, Vincristine, Prednisolone
Highly aggressive • Precursor lymphoblastic lymphoma/leukemia • Burkitt lymphoma – Tumor lysis syndrome – t8;14, t8;22 Treatment: CODOX-M cyclophosphamide, vincristine, doxorubicin, methotrexate
Salvage therapy • ESHAP – Etoposide, methylprednisolone, cytarabine, cisplatin
• ICE – Ifosfamide, carboplatin, etoposide
HIV-related brain lymphoma • CD4< 50 • Standard- radiotherapy • HAART … as soon as possible
Aggressive T cell • Adult T cell leukemia: HTLV-1 – Skin rash, LN,L+S+, Ca!, LDH! Tx: Doxorubicin based
• Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) – Middle age, elderly – Pruritic skin – Polyclonal hypergammaglobulin – Hemolytic anemia Tx: steroids, CMT
Multiple myeloma • Monoclonal: G>L>A>D • DDX M protein plasma cell symptom – MGUS: <3 g/dl – SMM: >3 – Nonsecrete: <3
<10% >10 >10
+
Initial evaluation
Staging
No maintenance therapy
Drugs • IV bisphosphanate: all patients – Tx hypercalcemia – Osteoporosis – Skeletal complications – Improve QoL
• Bortezomib: proteosome inh • Thalidomide: s/e DVT, neuropathy
Solitary Plasmacytoma • BM- negative • TX local radiation
MDS • 65-70 yr • Blood smear: macrocytosis, hypogranular neutrophils, pseudo Pelger-Huet • BM: dysplastic, ringed sideroblast, micromegahypolobated • Cytogenetics: 5q-, -7, trisomy 8
Treatment 1. Performance status Poor- transfusion, supportive care
2. Risk Low/inter: Epo, lenalidomide..5q-, azacytidine Inter/high: young HSCT azacytidine, combination CMT
Hypoplastic MDS: CsA, ATG
PRCA • • • •
Thymoma, CNT, MG, CLL, LGL, 5qParvovirus B 19 Erythropoietin, chemotherapy HIV
• BM: giant pronormoblasts parvovirus uninuclear micromega 5q-
Treatment • • • • •
Steroid- moderate dose Immunosupress- CsA, ATG Rituximab Thymoma surgery Parvovirus IVIg
Polycythemia vera • • • • • •
Hypertension Thrombosis Pruritus– post bathing Erythromelalgia Joint pain Visual disturbance
Bone marrow • BM: hypercellularity, trilineage hyperplasia, megakaryocyte hyperplasia, dec iron
Diagnostic criteria, 2008 WHO • • • •
Male > 18.5, female >16.5 JAK2 V617F Subnormal EPO BM trilineage
Treatment • All patients – Phlebotomy: Hct male<45%, female <42% – Low dose ASA
• Inter/high risk: age>60, prior VTE, plt> 1.5 mil – hydroxyurea
Essential thrombocytosis • Platelet> 450,000
