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board review > board review heme > Flashcards

board review heme Flashcards

1
Q

Lymphoma sx

A

B sx
adenopathy>6 weeks
splenomegaly
immune deficiency
autoimmune phenomena

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2
Q

lymphoma workup

A

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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3
Q

lymphoma workup

A

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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4
Q

hodgkin lymphoma

A

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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5
Q

tx hodgkin

A
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6
Q

toxicity with HL tx

A

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

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7
Q

non-hodgkin lymphoma

A

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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8
Q

CLL

A

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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9
Q

tx in CLL

A

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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10
Q

marginal cell lymphoma

A

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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11
Q

mantle cell lymphoma

A

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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12
Q

DLBCL

A

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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13
Q

burkitt lymphoma

A

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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14
Q

NHL post transplant lymphoproliferative disease

A

lymphaednopathy associated with polyclonal cells

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15
Q

primary CNS lymphoma

A

HIV
initial tx with IT methotrexase
relapse deisease tx with radiation

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16
Q

T cell lymphoma

A

treat like other aggressive lymphomas/

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17
Q

cutanous t cell lymphoma

A

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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18
Q

cutaneous t cell lymphoma

A

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

19
Q

MGUS

A

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

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

20
Q

smoldering MM

A

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

21
Q

plasmacytoma

A

single plasma cell lesion
tx with rad

22
Q

MM

A

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

23
Q

MM

A

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***

24
Q

M spike

A

POEMS
cryo

25
Q

Lymphoplasmacytic lymphoma/Waldenstroms

A

hyperviscosity sx
tx with ritux and plasampheresis

manifestations
hyperviscosity
peripheral neuropathy
cold agglutinin diseasse
cryo
amylod deposition

26
Q

amyloid

A

tx essentially as MM if 2/2 light chains

27
Q

bortezonib

A

neuropathy

28
Q

lenalidomide

A

thrombosis, bad in pregnancy

29
Q

bortezomib

A

neuropathy

30
Q

MDS

A

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

31
Q

5q- syndrome***

A

anemia, thrombocytosis
tx: lenolidomide

32
Q

CMML

A

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

33
Q

AML

A

> 20% blasts for AML
complications:
–infection, bleeding/DIC
–leukostasis –> cardiopulmonary or CNS
—–tx: leukpophresis

tx induction chemo, consolidation, allogenic transplant if possible
TLS risk

34
Q

acute promyelocytic leukemia

A

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

35
Q

acute promyelocytic leukemia

A

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

36
Q

acute promyelocytic leukemia

A

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

37
Q

ALL

A

> 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

38
Q

ALL complications

A

often curable

secondary cancer
heart disease
metabolic syndrome
osteopenia

39
Q

hairy cell leukemia

A

massive splenomegaly
infection/neutropenia risk

previously TRAP positive now CD11c, CD103 positive
single round of cladribine can be curative

40
Q

large granular lymphocytic leukemia

A

splenomegaly, B sx, RA, neutropenia
-if no LHL, feltys syndrome
cmv associated

tx: cyclophosphamide and mtx

41
Q

CML

A

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

42
Q

primary myelofibrosis

A

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

43
Q

stem cell transplant

A

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

44
Q

cytokine release syndrome

A

edema, fever, hypotension
neuro changes – catatonia?

tx: tociluzimab (IL-6 inh)

board review (11 decks)

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