MPNPresentation Diagnosis Labs/Cytogenetics Images Treatment

CML splenomegaly t(9,22) Philadelphia chromosome

High WBC, high/preserved platelets, low emoglobin

Tyrosine kinase inhibitor

PVPMFETNon-Hodgkin & Hodgkin LymphomaB cell Non-Hodgkin Lymphomas (B-cell NHL)

All CD20+; treat w/ rituximabFollicular lymphomaChronic lympholytic leukemia/small lymphocytic lymphoma (CLL/SLL)Marginal Zone Lymphoma (MZL)Hairy cell leukemia (HCL)Aggressive B-cell

non-hodgkin lymphomasDiffuse large B cel lymphoma (DLBCL)Mantle cell lymphoma (MCL)Burkitt’s lymphoma/leukemia

Starry skyLymphadenopathy

t(8,14) => incr c-myc expression

EBV in almost all African cases, but only 50% US

Starry sky Like AL, monitor/prophylaxis for tumor lysis

Like ALL, CNS prophylaxis

Primary CNS lymphoma (PCNSL)Post-transplant lymphoproliferative disorder (PTLD)

T-cell Non-Hodgkin Lymphomas (T-cell NHL)Hodgkin Lyphoma (HL)

CD15 & CD30 (CD20 neg)

Reed-Sternberg cells (bi-nucleat cell)

Myelodysplastic BM Cellularity Use history & Dysmyelopoiesis/ Goals: restore marrow fn; delay progression to

Syndromes (MDS) (+/-10%) = 100-age

Median age dx: 65 yo

Incidental finding on CBC

Lineage-specific symptoms:Leukopenia: recurrent infxn, poor wound healing

Anemia: fatigue, chest pain, shortness of breath w/ rest/exertion, dizziness

Thrombocytopenia: easy bleeding, bruising

Constitutional symptoms: fevers, night sweats, weight loss

labs to rule out other causes; if CBC abnormalities persist, order flow cytometry & cytogenetics/FISH

Hypercellular BM

Blasts <20% (otherwise, AML)

Peripheral blood cytopenia(s)

Dysplastic, dysfunctional cells

dysgranulopoiesis: abnormalities in nuclear segmentation – hyposegmented (1-2 lobeS) or hypersegmented (>5-6 lobes)

abnormal granulocytes (eosinophil, basophil); may show vacuolated cytoplasm

AML

Treatment varies based on prognosis:• Low-risk à transfusion support,

growth factors, antibiotics• Higher-risk, but frail à low-intensity

chemotherapy• High-risk, good performance status à

aggressive leukemia-like chemotherapy, stem cell transplant

DyserythropoiesisAbnormality in nuclear contour; multi-nuclearity

Dyssynchrony: discrepancy btwn nuclear & cytoplasmic maturation

Megablastic changes: abnormally large precursors

Cytoplasmic/basophilic stippling (also seen with lead poisoning)

Prussian blue stain for iron: iron abnormally localized in mitochondria & forms ring around nucleus => ringed sideroblasts

DysmegakaryopoiesisHypolobation, separation of nuclear lobes, abnormally small megakaryocytes

Abnormal bone marrow (fat replaces marrow)

Acute Myeloid Leukemia (AML)

Blast fraction greater than or equal to 20%

BMB: >20% marrow cells comprising myeloblasts; hypercellular (often 100%)

Cytogenetic abnormalities:

Platelet count low (thrombocytopenia), mild to severe anemia

High (leukocytosis) or low (leukopenia) WBC (WBC n: 4,000-10,000 cells/uL), up to 600,000

Neutropenia

WBC differential: blasts

Auer rods (myeloperoxidase granules in cytoplasm) in myeloblasts

myeloblast

1) Induction (goal = remission = peripheral blood counts restored; neutrophils >1000 cells/uL, Hb>10g/dL, platelets>100,000/uL, BM <5% blasts); 7+3 (&d cytarabine + first 3d idarubicin)

Day 14: repeat BMB to assess response to therapy

Day 28-30: BMB when peripheral blood counts recovered

2) Consolidation: chemo; if curate rate with chemo alone poor, then allogeneic bone marrow transplant

Leukopheresis to reduce WBCs/blasts (goal: get blast count <50,000) & prevent leukostasis

End goal: restore normal hematopoiesis

Watch for tumor lysis syndrome

Acute promyelocytic leukemia with t(15;17)(q22;q12)

Translocation btwn PML & RARA gene => Cells cannot mature beyond the promyelocyte stage given the abnormal fusion protein and subsequent block of transcription

Promyelocytes instead of blasts, with slightly clefted/bilobed nucleus & prominent cytoplasmic granules, multiple Auer rods

High risk of DIC – low fibrinogen, elevated D-dimer/PT/aPTT, thrombocytopenia, schistocytes on smear

All-trans retinoic acid (ATRA) & arsenic to dissolve fusion protein

Acute Lymphoblastic Leukemia (ALL)Subtypes: precursor

Flow cytometry for TdT to test for lymphoblasts,

25% of adult ALL: t(9;22) BCR-ABL Philadelphia chromosome

CNS prophylaxis with intrathecal and/or systemic chemotherapy to penetrate CNS and/or craniospinal irradiation

B (~80%), precursor T

and categorize subtypes

Maintenance therapy: prolonged courses of low-dose chemotherapy

Philadelphia chromosome+ ALL: tyrosine kinase inhibitors (imatinib or dasatinib)

InductionConsolidation-Intensification (just chemo)

Allogeneic stem cell transplant for pts predicted to have low cure rate with just chemo

Prognostic factors for each;Normal lab values & morphology