board review heme Flashcards by H L

Lymphoma sx

B sx
adenopathy>6 weeks
splenomegaly
immune deficiency
autoimmune phenomena

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lymphoma workup

ultrasound of nodes
excisional bx > FNA

PET/BMBx/LP will depend on lymphoma. If more aggresive, will need PET.
Can forego BMBx if PET ok/no cytopenias/flow neg

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lymphoma workup

ultrasound of nodes
excisional bx > FNA

PET/BMBx/LP will depend on lymphoma. If more aggresive, will need PET.
Can forego BMBx if PET ok/no cytopenias/flow neg

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hodgkin lymphoma

presentation: adenopathy +/- B sx/itching
classical - CD15/CD 30 Reed-sternberg cells
-nodular lymphocyte predominant HL- neg CD15/CD 30 but CD 20+

dx: excisional bx, flow generally neg
pet and bone marrow bx for staging

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tx hodgkin

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toxicity with HL tx

bleomycin lung toxicity, cardiomyopathy with Adriamycin,
secondary malignancies
chest radiation –>cv issues
hypothyroidism
referral for fertility

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non-hodgkin lymphoma

follicular lymphoma-indolent
-asx patients often monitored

BCL-2 over-expressed by t(14:18)***
CD19/CD20 (B cell), CD10+ (follicular center)

grade I/II/IIIA-radiation v ritux+/- chemo
risk of transformation to aggressive DLBCL

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CLL

may be indolent, common
immunocompetent cells, often with immune dysregulation
CBC will show >5k lymphocytes, “smudge cells” (<5k is MBL)

flow cyto with CD5+ cells, may not need staging scan or BMBx

high risk: 17p-, 11q-
low risk: 13q, IGHV mutated

transformation more rare

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tx in CLL

treatment for anemia/thrombocytopenia/bulky dz
may respond to course of chemo+antiCD20
- venetoclax (oral) v bendamustine

non-chemo candidtes can try acalibrutinib and ibrutinib (increase bleeding risk)**

high risk for infections! hypogammaglobulinemia, B and T cell dysregulation*

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marginal cell lymphoma

CD20+, CD10_, infiltrative small lymphocytes
nodal - may be curable with local radiation. If not may be observed

**MALT/extranodal – routinely associated with infections
- H pylori in GI tract, campylobacter, thyroiditis, chlamydia psittaci in eye

splenic (associated with HCV) may be observed, surgical if symptomatic or cytopenic.

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mantle cell lymphoma

t(11:14)
proliferative index (Ki67>30%) is prognostic
frequently seen in GI tract

tx with lymphoma regimens with R or R-CHOP)

mantle cell transplant

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DLBCL

most common lymphoma
MYC/BCL-2/BCL-6 “double or triple hit” depending on if 2 or 3 mutations.

curative intent

tx:
RCHOP

if refractory: R-ICE and consider auto transplant

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burkitt lymphoma

highly aggressive B-cell germinal center
Myc translocation t(8;14)
“starry sky”
ki67% > 95
strong association with EBV (EBER staining)
endemic form usually in jaw
outside of Africa, often seen in abdomen

also seen in HIV+

high risk TLS -consider G6PD testing prior to starting rasburicase

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NHL post transplant lymphoproliferative disease

lymphaednopathy associated with polyclonal cells

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primary CNS lymphoma

HIV
initial tx with IT methotrexase
relapse deisease tx with radiation

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T cell lymphoma

treat like other aggressive lymphomas/

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cutanous t cell lymphoma

mycosis fungoides/sezary
presenting: may be erythroderma, plaques, macular-papular, pruritus

systemic/sezary: GI, lung, nodal

local tx for skin: electron beam radiation, extracorporeal photopheresis, retinoids, purine analogues

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cutaneous t cell lymphoma

mycosis fungoides/sezary
presenting: may be erythroderma, plaques, macular-papular, pruritus

systemic/sezary: GI, lung, nodal

local tx for skin: electron beam radiation, extracorporeal photopheresis, retinoids, purine analogues

MGUS

<10% marrow, no CRAB criteria, protein <3g

IgG is low risk ~1 %developing
hold BMBx?
monitor q6 months

smoldering MM

> 10% marrow or M-spike >3g
tx controversial

plasmacytoma

single plasma cell lesion
tx with rad

CRAB criteria
elevated free Light chain ratio 1:100
dx: SPEP, UPEP, free light chain ratio
bone surveys v PET

tx: dex, lenalidomide, bortezomib, ASA, bisphosphonate
consider auto transplant when in remission, continue lenalidomide

CRAB criteria
elevated free Light chain ratio 1:100
dx: SPEP, UPEP, free light chain ratio
bone surveys v PET

tx: dex, lenalidomide, bortezomib, ASA, bisphosphonate
consider auto transplant when in remission, continue lenalidomide
-allo
- CAR-T

note risk of thrombosis with lenalidomide so put on aspirin***

M spike

POEMS
cryo

Lymphoplasmacytic lymphoma/Waldenstroms

hyperviscosity sx tx with ritux and plasampheresis manifestations hyperviscosity peripheral neuropathy cold agglutinin diseasse cryo amylod deposition

amyloid

tx essentially as MM if 2/2 light chains

bortezonib

neuropathy

lenalidomide

thrombosis, bad in pregnancy

bortezomib

neuropathy

MDS

hypercellular marrow with dysplasia variably progressive teardrop cells dohle bodies without infection giant platelets hyper or hypolobed granulocytes macrocytosis sweet syndrome-- neutrophilic dermatitis, fever tx" growth factors for low risk hypomethylating agents chronic transfusions ppx antibiotics? allo stem cell transplant if young hypoplastic MDS may need IS as looks like aplastic anemia

5q- syndrome***

anemia, thrombocytosis tx: lenolidomide

CMML

persistent peripheral monocytosis, splenomegaly, high risk for AML***

AML

>20% blasts for AML complications: --infection, bleeding/DIC --leukostasis --> cardiopulmonary or CNS -----tx: leukpophresis tx induction chemo, consolidation, allogenic transplant if possible TLS risk

acute promyelocytic leukemia

PML-RARa mutation t(15;17)responds to all-trans retinoic acid

acute promyelocytic leukemia

PML-RARa mutation t(15;17)responds to all-trans retinoic acid

acute promyelocytic leukemia

PML-RARa mutation t(15;17)responds to all-trans retinoic acid. also give arsenic trioxide. -treat when suspected as reduces dic risk - differentiation syndrome -- give steroids

ALL

>20% blasts in bone marrow +/-mass lesion B and T cell - stain for TdT -t cell may have mediastinal mass -b cell more common 75% often with cns involvement ph+ have t(9;22) mutation and carries higher risk philadelphia chromosome/imatinib

ALL complications

often curable secondary cancer heart disease metabolic syndrome osteopenia

hairy cell leukemia

massive splenomegaly infection/neutropenia risk previously TRAP positive now CD11c, CD103 positive single round of cladribine can be curative

large granular lymphocytic leukemia

splenomegaly, B sx, RA, neutropenia -if no LHL, feltys syndrome cmv associated tx: cyclophosphamide and mtx

CML

multiple myeloid forms, often >!00k, associated with anemia, thrombocytosis, and massive splenomegaly BCR-ABL1 fusion gene t(9;22) usually p210 chronic --> accelerated-->blast crisis tx: imatinib -monitor BCR-ABL burden -allogenic transplant if fails TKI therapy

primary myelofibrosis

fatigue, anorexia, splenomegaly, depression leukperythroblalstosis on smear (immature forms, teardrops) curative allogenetic SCT is curative otherwise ruxolitinib -- Jak inh interferon alpha splenic radiation supportive transfusions

stem cell transplant

auto high-dose chemo with rescue with previously collected stem cells received the following days infection risk (neutropenia), transfusion needs, mucositis allo-transplant may be umatched unrelated donors or related with HLA typing allows for graft v disease effects risks include above plus ***GVHD***, PTLD, vasocclusive disease of the liver, and associated need for long term IS acute GVHD -- GI, skin, liver chronic - above + msk/sclerodermic changes and lung disease

cytokine release syndrome

edema, fever, hypotension neuro changes -- catatonia? tx: tociluzimab (IL-6 inh)