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ASCO Boards > Malignant Heme > Flashcards

Malignant Heme Flashcards

1
Q

AML poor risk name 4 t( , )

A

6;9, 11q23, 9;22, 8;16

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2
Q

AML poor risk name 4 deletions/inversions

A

inv3, del5q or -5, -7, -17

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3
Q

wt NPM1 and FLT3 high AML risk

A

high risk

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4
Q

AML poor risk mutations (3)

A

RUNX1, ASXL1, p53

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5
Q

mut NPM1 and FLT3 high AML risk

A

intermediate

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6
Q

wt NPM1 FLT3 low AML risk

A

intermediate

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7
Q

intermediate risk translocation AML (1)

A

t(9;11)

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8
Q

favorable risk AML (4)

A

t(8;21), inv 16, mut NPM1 alone or FLT3 low, biallelic CEBPA

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9
Q

mut NPM1 with FLT3 low AML risk

A

low

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10
Q

patient gets 7+3 but too sick for transplant, medication for maintenance

A

azacitazine pills improves OS

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11
Q

AML 2 years after chemo name the drug and abnormality

A

topoisomerase or anthracycline; 11q23

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12
Q

AML 5-7 years after chemo and name 3

A

alkylating agent (cyclophosphamide, busulfan, melphalan)

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13
Q

Hepatic sinusoidal obstruction syndrome (SOS or VOD), symptoms and timeline, US finding, treatment

A

RUQ pain, ascites, hepatomegaly, elevated bilirubin, weight gain suddenly
<3 weeks from transplant
US liver shows reversed portal flow
Treat with defibrotide and supportive care

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14
Q

vaccine timing post transplant

A

inactivated upfront
live vaccine 2 years post-transplant

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15
Q

how many days post transplant defines chronic GVHD

A

100 days

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16
Q

compare and contrast GVHD related lung disease (2 types)

A

COP- GGO, restrictive PFTS, reduced DLCO, acute or chronic GVHD, treated with steroids

BO- bronchiolitis obliterans, chronic GVHD, narrowing of airways due to fibrosis, obstructive PFTs, treat the underlying GVHD

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17
Q

Chronic GVHD treatments (top 2 then others)

A

1) Steroids
2) Ruxolitinib JAK2 inhibitors

ibruitinib, alemtuzumab, belumosudil (ROK2 inhibitor), abatacept and others

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18
Q

gilteritinib, what for, timing of treatment, monitoring for 3 severe issues

A

FLT3 disease relapsed/refractory, differntiation syndrome, QTc, PRES

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19
Q

which drug is for IDH1 and which is for IDH2, side effect to worry about

A

IDH1 is ivosidenib
IDH2 is enasidenib

Differentiation syndrome

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20
Q

Which anti-rejection drug for transplant causes PRES

A

tacrolimus

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21
Q

treatment for blastic plasmacytoid dendritic cell neoplasm and MOA

flow findings

A

tragraxofusp is an anti-CD123 drug

CD4+ CD56+ CD123+ TCL1+

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22
Q

venetoclax and antifungal azoles, what should you do

A

dose reduce significantly

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23
Q

name a myeloablative conditioning regimen

A

Cy/TBI
Bu/Cy
BEAM (lymphomas auto)
Melphalan
CBV (carmustine, etoposide, Cy)

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24
Q

who gets myeloablative conditioning regimen

A

fit and healthy, hematologic malignancies not in CR, autologous transplant

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25
Q

name a non-myeloablative conditioning regimen

A

fludarabine/TBI

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26
Q

name a reduced intensity conditioning regimen (RIC) and who gets these

A

Coborbid conditions with good upfront disease control

FLu/Mel
flu/Bu
flu/cy
flu/Bu/thiotepa
flu/treo

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27
Q

CLL criteria to treat

A

symptoms (weight loss, fever, night sweats, debilitating fatigue)
marrow failure to stage III or IV rai
massive splenomegaly or nodes (very enlarged palpable, >10 cm nodes )
progressive lymphocytosis >50% over 2 months or LDT <6mo
autoimmune complication not responsive to steroids
extranodal involvement

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28
Q

CLL good risk

A

13q alone (charlie coyle), IGHV mutated

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29
Q

CLL intermediate risk

A

+12 or normal cytogenetics

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30
Q

CLL poor risk

A

del17p, del11q, IGHV unmutated, high b2 microglobulin, p53 sequenced, complex karyotype

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31
Q

p53 positive CLL responds best to

A

BTK inhibitors, venetoclax +/- obinutuzumab

They do not respond well to chemotherapy

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32
Q

name a PI3K inhibitor approved for use in CLL and what is the indication

A

duvelisib, idelalisib

cause colitis, pneumonitis, hepatotoxicity

3rd line therapy

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33
Q

hairy cell leukemia flow and treatment, common mutation

relapse time after which you could rechallenge

when do you check for response with marrow after treatment

A

CD19/20+, CD103+, CD11c+

cladribine or pentostatin

BRAF V600E (vemurafenib)

relapse within 2 years, change therapy

4 months after therapy with cladribine, pentostatin sooner (after count recovery)

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34
Q

Who is offered FCR for CLL and what side effect is concerning

A

young, fit with IGHV mutated for goal of long remission

fludarabine causes AIHA

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35
Q

drug to avoid in CLL with bleeding history or PUD or afib

A

ibrutinib

the other BTK (acala and zanubrutinb are supposed to have less bleeding and afib)

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36
Q

flow and differentiation of CLL from mantle cell lymphoma

A

CLL: CD5+ CD23+, CD20 low
Mantle: CD5+ CD20+ CD 23 neg

Cyclin D1 differentiates as it is always positive

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37
Q

ibrutinib, acalabrutinib, zanubrutinib resistance mutations

drug to overcome resistance

A

C481 mutation of the BTK kinase (grzekcyk)

pirtobrutinib

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38
Q

which CLL drug must you hold before surgery and for how long

A

BTKs and hold for 7 days before and after

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39
Q

TLS risk stratification in CLL starting venetoclax

A

outpatient if LN<5 cm and ALC <25k

outpatient with IVF if LN 5-10 cm or ALC<25

inpatient if LN >10 cm or if LN 5-10 and ALC >25

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40
Q

CLL treatment: venetoclax with which drug is first line and which drug is second line

A

Ven+ obinutuzumab is first line
Ven+ rituximab is second line

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41
Q

CLL related hematologic entities

A

AIHA
Pure red cell aplasia
ITP
Hypogammaglobuliemia

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42
Q

B-cell prolymphocytic leukemia (B-PLL), immunophenotype and treatment

A

CD20/19+, CD22+ CD79a+, FMC7+, IgM+, IgK or L+

if p53 + treat with BTK

FCR, BR or BTK inhibitors

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43
Q

venetoclax uptitration plan in CLL

A

20 mg for 1 week, 50 mg for one week, 100 mg for 1 week, 200 mg for 1 week, 400 mg thereafter

TLS prophylaxis with hydration and allopurinol

if TLS, hold the dose for 1-2 days, give IVF, repeat labs and if resolved resume same dose

if takes >48 hours to resolve, then go down a dose

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44
Q

ibrutinib interactions

A

cyp3a

avoid grapefruit

do not use if they are on HIV meds that are strong CYP3A inhibitors

dose reduce to 140 mg if moderate inhibitor must be used like fluconazole, voriconazole, diltiazem, verapamil, ciprofloxacin

420 is the normal dose

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45
Q

erythroid CD markers

A

CD71 and CD235a

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46
Q

myeloid CD markers

A

cMPO, CD13, CD33, CD117, CD15

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47
Q

stem cell markers CD

A

CD34, HLA-DR, TdT

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48
Q

monocyte CD markers

A

CD64, CD14, CD11b, CD11c, lysozyme

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49
Q

megakaryocyte cd markers

A

CD41 (GPIIb/IIIa), CD61, CD36, CD42b

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50
Q

B lymphoid cd markers

A

CD19, CD20, CD10, CD22, CD79a

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51
Q

T lymphoid markers

A

CD3, CD5, CD7, CD1a, CD2, CD4, CD8

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52
Q

NK CD marker

A

CD56

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53
Q

APML flow findings

A

CD33+ bright CD13 +, negative CD34 and HLA-DR

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54
Q

First line treatment CML low risk

A

Imatinib (400 mg) or 2nd gen TKI

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55
Q

First-line treatment CML int or high-risk

A

2nd gen TKI
nilotinib (300), dasatinib (100), bostutinib (400)

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56
Q

definition of accelerated phase CML

A

MDACC modifiend criteria
peripheral myeloblasts >15% but <30 %
peripheral pro and myeloblasts >30%
peripheral basophils >20%
thrombocytopenia with count <100k
other cytogenic abn other than just ph+ (trisomy 8, 17q, trisomy 19, ch3 abn

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57
Q

treatment for accelerated phase CML

A

2nd generation TKI (higher doses than chronic phase) or 3rd gen

or

omacetaxine if progression from CP-CML

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58
Q

define blast phase CML and treatments

A

peripheral or marrow blasts >30% or extramedullary CML
AML or ALL induction with TKI depending on whether myeloid or lymphoid differentiation

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59
Q

BCR-ABL level should be where after 3-6 months, if not what should be done

A

at least <10%, if not evaluate adherence, drug interactions, mutation testing, consider BMB. Switch to another TKI. Eval for allogenic HCT.

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60
Q

If BCR-ABL level is not ____ by 12 months, what should be done

A

<1%, if not then evaluate adherence, drug interactions, mutation testing, consider BMB. Continue with close monitoring or switch to another TKI.

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61
Q

Goal of BCR-ABL level with treatmet

A

<1% optimal for LT survival
<0.1 for long treatment-free survival (pre-requisite for a trial off of TKI)

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62
Q

CML mutation T315I, TKI choice

A

use ponatinib, asciminib, omacetaxine

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63
Q

Bosutinib mutations to avoid

A

V299L, G250E, F317L

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64
Q

Dasatinib mutations to avoid

A

V229L or F317

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65
Q

nilotinib mutations to avoid

A

Y253H, E255K F359

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66
Q

asciminib mutations to avoid

A

A337T, P456S

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67
Q

CML MMR versus MCyR

Define relapse

A

MMR= major molecular response is BCR-ABL level <0.1

MCyR= mayor cytogenetic response is Ph positive metaphases <35%

loss of hematologic response (cytopenias), loss of CCyR or BCR-ABL >1%, 1 log increase in BCR-ABL transcript level

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68
Q

Criteria for TKI discontinutation trial

Indication to restart treatment while on trial

A

chronic phase CML only, TKI for 3 years, stable molecular response with BCR-ABL <0.01% for >2 years

monthly monitoring for 6 months, then space out moving forward

loss of MMR BCR-ABL >0.1 should prompt restarting the TKI

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69
Q

Nilotinib risks/se

A

Qtc monitoring, vascular events (peripheral arterial), pancreatitis

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70
Q

bosutinib risks/se

A

diarrhea, liver toxicity

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71
Q

dasatinib risks/se

A

pleural effusions, pulmonary hypertension

72
Q

imatinib risks/se

A

fluid retention, GI upset, muscle cramps, rash

73
Q

ponatinib risk/se

A

hemorrhage, cardiac arrythmia, CHF, fluid retention, HTN, liver toxicity, pancreatitis, rash

74
Q

omacetaxine MOA and use

A

protein synthesis blocker
Approved for use in CP-CMP with T315I mutations, or accelerated phase CML that progressed from CP-CML. Or ne-novo AP-CML with resistance to or intolerance to two or more TKIs.

75
Q

CML pregnancy risks

A

TKIs are teratogenic and cannot be given during pregnancy

Patient must be stable on TKI for 3 years with CMR for 2 years prior to safe stop per trials

If BCR-ABL increases to >1% then treatment should be resumed but if pregnant, interferon alpha can be given to try to keep CML under control

76
Q

asciminib risks/se and use

A

CP-CML treated with 2 other TKIs or CP with T315I mutation

elevated amylase and lipase/pancreatitis, hypersensitivity, HTN

77
Q

Ponatinib dose reduction

A

start at 45 mg daily then if BCR-ABL of 1% or less, reduce the dose to 15 mg daily which reduces side effects

78
Q

HL stage and risk strat

A

I one site, II multiple sites only above or below, III multiple sites above and below, IV marrow or extranodal disease

unfavorable risk factors include:
- ESR>50 or >30 if B sx
- Mediastinal mass ratio >0.33
- >3 nodal sites
- bulky disease >10 cm node

79
Q

Early-stage unfavorable HL treatment

A

ABVD x2–>PET–>deauville 1-3–> 2 more ABVD and ISRT OR just 4 cycles AVD (RATHL)

if deauville 4-5 escalate to BEACOPP x2 –>PET deauville 1-3 BEACOPP x2

if deauville 4-5 after BEACOPP then refractory disease

80
Q

late stage HL treatment
also describe the new regimen protocol

A

ABVD–>PET with deauville 1-3–> AVD x4

ABVD–>PET with deauville 4-5–> BEACOPP x3–>PET–>good–> 1 more BEACOPP

Also BV+ AVD x6 (ECHELON-1) and restage with PET (1-3 observe), 4-5 biopsy.

81
Q

early stage non-bulky HL

A

ABVD x2 and ISRT 20 if no high-risk features

ABVD x2–>PET–>deauville 1-3–> 1-2 more ABVD (D1-2 vs D3) and ISRT 30 OR just 4 cycles AVD (RATHL)

if dauville 4–> 2 more ABVD–>PET–> good then radiate

if dauville 5 biopsy

82
Q

HL in pregnancy treatment

A

ABVD can be given after the 1st trimester

83
Q

siltuximab MOA and use

A

antibody against IL-6 used in castlemans disease that is HHV8 negative

if positive, treat with rituximab

84
Q

mycosis fungoides/sezary syndrome what is it and how do you treat it by stage

A

cutaneous T cell lymphoma, sezary is the late stage with blood involvement and lymphadenopathy

IA minimal skin only-local therapy
IB/IIA skin only with >10% BSA skin or skin and systemic

IIB limited tumors-local RT and skin therapy or systemic and RT

III erythema covering 80% BSA treat with systemic and skin

IV is sezary syndrome lymph and blood inv

systemic treatments include mogamulizumab (CCR4 blocker), BV (second line), romidepsin, interferon alpha, MTX, dexarotene, ECP

85
Q

maintenance after autotransplant in HL criteria

A

BV if 2 or more

remission less than 1 year
extranodal disease
PET positive at the time of transplant
B sx
>1 salvage regimen

86
Q

HL approved as second-line treatments or general R/R disease

A

BV +/- benda or nivo
DHAP
ICE +/- benda or nivo
pembro +/- ICE
GBV

87
Q

HL only approved in the third-line treatments

A

bendamutstine
everolimus
lenalidomide
nivolumab

88
Q

treat MDS with 5q

A

treatment with lenalidomide in low or int risk MDS

89
Q

treat CMML with 5q32 translocation aka PDGFRB gene rearrangement or t(5;12) ETV6-PDGFR beta fusion

A

treat with imatinib

eosinophilia

90
Q

treat hypoplastic MDS with HLA-DR15

A

treat with ATG and cyclosporine

91
Q

AML defining mutations

A

t(8;21), inv16, t(15;17)

92
Q

high risk MDS treatment

A

azacitadine or decitabine or decitabine+cedazuridine(inqovi oral drug)

93
Q

mutation associated with MDS with ringed sideroblasts

A

SF3B1, higher platelet count, higher neutrophil counts, and longer EFS compared to WT

94
Q

Atypical CML

A

lacks the BCR-ABL fusion, often mutated SETBP1 gene

95
Q

MDS prognostic risk groups by cytogenetics

A

good: -Y, 5q, 20q-, normal
int: other not classified
poor: ch 7 abn and >3 abn

in R-IPSS
del11q is very good
good includes 12p
int includes trisomy 8 and 19
poor includes ch3 issues

96
Q

treat acute GVHD

A

steroids, methotrexate, cyclosporine

next line ruxolitinib and ATG, alemtuzumab, infliximab, etanercept, sirolimus, cellcept, tocilizumab, pentostatin, calcineurin inhibitors

97
Q

childhood MDS mutations

A

SAMD9L monosomy 7, GATA2, CEBPA, RUNX1, DDX41, ETV6, ANKRD26

98
Q

prevent acute GVHD meds

A

ppx with cyclosporine and mtx
abatacept T cell modulator for prophylaxis of GVHD in combination with a calcineurin inhibitor and MTX

98
Q

mutations prognosis in MDS

A

good SF3B1
poor ASXL1, EZH2, SRSF2, U2AF1, ZRSR2, RUNX1, TP53, STAG2, NRAS. ETV6, GATA2, IDH2, BCOR, FLT3, WT1, NPM1

99
Q

Juvenile myelomonocytic leukemia (JMML) treatment

A

azacitadine

99
Q

myeloma definition

A

plasma cells >10% + one

elevated calcium
renal insufficiency cr>2
anemia hgb<10 or <2 below LLN
bone lesion
Free light chain ratio >100

or plasma cells >60%

99
Q

decitabine is particularly useful in MDS with which mutation

A

p53

100
Q

smoldering myeloma definition, treat

A

M-spike >3
Bence-jones >500/24h
marrow plasma cells 10-59%
absence of myeloma-defining events

observation versus PERTHEMA-GEM suggestion of survival benefit from treatment of high risk with lenalidomide/dex (>20% plasma, M spike >2, free light chain ratio >20)

101
Q

myeloma staging

A

I= normal beta2 and albumin
II= low albumin
III= high beta 2

R-ISS:
I: ISS I and standard risk chromosomes, normal LDH
II: in between
III: ISS stage III, elevated LDH or high risk FISH

102
Q

high risk myeloma cytogenetics/FISH

A

t(4;14), t(14;16), t(14;20), 17p del, 1q21 gain or amp, MYC translocation, 13q, tetrasomies or complex karyotype

103
Q

DVT ppx in MM on revlemid

A

ASA 81-325 if <3 points on IMPEDE or 2 by SAVED, if more then start on anticoagulation ppx dose lovenox or DOAC

104
Q

zolendronic acid renal cut-off , what do you give instead

A

<30 do not give, give denosumab

105
Q

good risk cytogenetics/FISH in myeloma

A

trisomies, t(11;14), t(6;14), hyperdiploidy

106
Q

daratumumab moa

A

CD38 antibody

107
Q

elotuzumab moa and use

A

Antibody dependent cellular toxicity (ADCC) myeloma drug against SLAM7 which causes NK cells to kill myeloma cells.

for use in RR myeloma only in combination in third line

108
Q

belantamab mafodotin moa and use

A

myeloma anti-BCMA antibody conjugated to cytotoxic maleimidocaproyl monomethyl auristatin F, 4th line

109
Q

selinexor moa and use

A

myeloma selective inhibitor of nuclear export, relapsed or refractory DLBCL, 4th line

110
Q

teclistamab moa and use, side effect to worry about

A

myeloma BITE targeting CD3 and BCMA, CRS and neurologic se

111
Q

waldenstrom indications for treatment and treatment options, mutation

A

anemia or TCP, bulky adenopathy or HSM, hyperviscosity syndrome, neuropathy, amyloidosis, cryoglobulinemia, cold agglutinin disease or B symptoms.

treat with BR or RVd or ibrutinib +/- rituximab or zanubrutinib

myd88

112
Q

POEMS defined and required major criteria and other criteria for diagnosis

A

polyneuropathy (major), organomegaly, endocrinopathy, M-protein (major), skin changes

1 other: castlemans, sclerotic bone lesion or elevated VEGF

1 minor: organomegaly, volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis/polycythemia

113
Q

isatuximab moa and use

A

myeloma via antibody against CD38 in combination with pom and dex in relapsed refractory setting

114
Q

fedratinib moa and use and risk

A

myelofibrosis symptom management, JAK2 inhibitor, wernickes encephalopathy

115
Q

hypereosinophilic syndrome diagnosis and treatment

A

diagnose: eos >1500 for >6 mo, ruled out other causes, end organ involvement

molecular testing for PDGFRA, PDGFRB, FGFR1, PCM1-JAK2, chronic eosinophilic leukemia NOS

CHIC2 FISH

steroids, hydrea, JAK inhibitors, mepolizumab, alemtuzumab

targeted therapy with imatinib in those who have FIP1L1/PDGFRA or ETV6-PDGFRB (5q31-33)

116
Q

treat aggressive systemic mastocytosis

mutation and mast cell markers, symptoms

mast cell leukemia definition in comparison

A

CD117+ CD25+ C-KIT D816V

treat with midostaurin, avapritinib, inf-alpha, cladribine

early bone loss, diarrhea/flushing, rash, headache, HSM

leukemic form has marrow with 20% mast cells, poor prognosis

117
Q

primary myelofibrosis risk stratification

A

Dynamic International Prognostic Scoring System (DIPSS), MIPSS

based on age, constitutional symptoms, cytopenias, blast percentage

now they are adding karyotype and mutations

high risk–> eval for transplant

118
Q

chronic neutrophillic leukemia mutation and symptoms

A

CSF3R, mature neutrophilic leukocytosis and infiltration, HSM

119
Q

pacritinib moa and use

A

JAK2 inhibitor for myelofibrosis with plts <50k (int or high risk)

120
Q

langerhans cell histiocytosis flow markers, treatment

A

CD1a+, CD207+ dendritic cell markers

if multifocal disease if BRAF+ treat with vemurafenib, otherwise treat with cobimetinib,

no mutation can also treat with cytarabine or cladribine

121
Q

ET risk categories and treatment options

A

very low risk: <60 with no JAK2 or clot (observe unless symptoms)

low risk <60 with JAK 2 no clot (ASA usually)

int risk >60 no JAK2 no clot (ASA and hydrea)

high risk thrombosis or >60 with JAK2 (ASA and hydrea)

Second line includes interferon or anagrelide

extreme thrombocytosis is not in itself an indication for cytoreductive therapy. >1mill should screen for acquired vws and if present, treat with cytoreductive therapy

122
Q

PV risk strat and treatment

A

Low risk <60, no clot
high risk clot or >60

ASA for all, hydrea if high risk

second line treatment: peginterferon alfa-2b

123
Q

MPN with FGFR1 rearrangement

A

treat with pemigatinib (FGFR1 inhibitor)

124
Q

TEMPI syndrome what is it and symptoms, lab finding, treatment

A

telangiectasias, erythrocytosis, monoclonal gammopathy, perinephric fluid collection, intrapulmonary shunting

elevated EPO 10x500x

treated with bortezomib

125
Q

follicular lymphoma diagnosis

A

t(14;18), CD19/20+, CD10+, BCL-2

126
Q

flipi

A

age, hgb, node areas >4, ldh >uln, stage

127
Q

tazemetostat moa and use

A

EZH2 inhibitor for relapsed or refractor follicular lymphoma with an EZH2 mutation with 2 prior therapies or other options . acts as a methyltransferase inhibitor.

128
Q

Gastric MALT lymphoma with t(11;18) meaning

A

treat for h. pylori but also give radiation due to less benefit of hpylori treatment alone with this translocation

129
Q

splenic marginal zone lymphoma, markers and presentation, associated disorder and treatment

A

splenomegaly, pancytopenia but with lymphocytosis, marrow involvement (no nodes)

CD20+ CD22+, negative for CD5/25/10/103/cyclinD

NOTCH2 and KLR2 mutations

hep c associated–>treat it and lymphoma may regress

other treatment is rituximab or splenectomy

130
Q

Mantle cell treatment

A

Indolent CLL form: observe if asymptomatic

Nodal aggressive form:
- transplant candidate: induction chemo (TRIANGLE regimen R-CHOP+ BTKi/RDHA nordic regimen (maxi-CHOP and cytarabine/ritux) + auto transplant + rituximab maintenance +/- BTK maint

Not transplant candidate:
- BR (no maintenance)

2nd line:
- BTK inibitors like acalabrutinib
- lenalidomide ritux
- bortezomib ritux
- ven ritux or ven ibrutinib

3rd line:
- pirtobrutinib or carT (brexucabtagene autoleucel)

131
Q

Nasal NK/T treatment (19)

A

early stage: chemo/rads

often asparginase based like
SMILE regimen (steroid, methotrexate, ifosfamide, asparginase, etoposide)

132
Q

primary effusion lymphoma, associated condition, virus, treatment

A

HIV and HHV8
R-EPOCH

133
Q

t cell prolymphocytic leukemia translocation

A

inv(14)(q11q32), CD2/5/7/52 TCR+

134
Q

breast implant-associated lymphoma

A

ALCL anaplastic large cell lymphoma

total capsulectomy and implant removal bilaterally–> follow

incomplete excision, lymph nodes RT+systemic therapy with BV, CHOP etc

135
Q

plasmablastic HIV associated lymphoma treatment and markers

A

CD138 ++, neg CD20, often EBV +

EPOCH or HyperCVAD

IT chemo

136
Q

Post transplant lymphoproliferative disorder PTLD types and presentation and treatment

A

non-destructive lesions–> reduce intensity of immunosuppression in conjunction with transplant team, add rituximab if needed for partial response

monomorphic (B cell type) treat with rituximab and RI or chemo/immunotherapy such as R-CHOP

monomorphic T cell type: BV+ CHP or CHOP/CHOEP

137
Q

grey zone lymphoma markers

A

CD45/pax5/bob1/oct2/cd15/cd20/cd30/cd79a

138
Q

primary mediastinal BCL diagnosis markers and treatment

A

c-rel and TRAF-1

treat with R-EPOCH x6 versus R-CHOP+ radiation

139
Q

DLBCL treatment by stage

A

Stage I/II non bulky R-CHOP x3 with radiation or R-CHOP x4

Early stage bulky (>7.5 cm) then R-CHOP x6 +/- rads

III/IV R-CHOP x6

140
Q

burkitt translocation, immunophenotype, and treatment

A

t(8;14), (2;8), (8;22); cd20/cd22/cd10/bcl6
R-hypercvad + IT chemo

141
Q

adult T cell leukemia/lymphoma, virus, presentation, smear finding, immunophenotype, treatment

A

HTLV-1
osteolytic bone lesions with hypercalcemia, liver and skin involvement
CD2/3/4/5+ CD25+ TCR+

smoldering–> zidovudine/interferon

acute disease EPOCH or BV +cy+doxorubicin+pred

second line mogamulizumab

142
Q

Pulmonary lymphomatoid granulomatosis what is it and treatment

A

EBV asscoiated lymphoma with multiple pulmonary nodules with lymphocytic invasion of vascular walls on biopsy, granulomatosis.

Stop the immunosuppression medication, treat with chemo-immunotherapy if if is high grade (R-CHOP_

143
Q

marginal zone treatments

A

Nodal:
Stage I or II
- radiation and rituximab
Stage III or IV
- BR
- R-CHOP
- rituximab alone in elderly

second line
- acalabrutinib or len ritux

3rd line
- copanlisib or car-t anti cd19(axicabtagene cilolucel)

144
Q

cart bridge for rr dlbcl

A

if avaliable, preferred to switch over second line chemo like DHAP

145
Q

ALL post-induction consolidation

A

if CR with MRD-:
- then consolidate with blinatumomab +/-TKI then maintenance therapy
- or consolidate and then transplant if high risk with post transplant TKI

if CR with MRD + then allogeneic transplant indicated.
- consolidate with blinatumumab + TKI or TKI beforehand
- post-transplant TKI

if no CR, then relapsed/refractory treatment

146
Q

ALL induction treatment stratification

A

AYA vs Adult

Ph+ or negative

Always give IT chemo ppx!

Elderly:
- TKI + steroid +/- vincristine
- steroid and vincristine or POMP
- blinatumomab +/- TKI

147
Q

blinatumomab moa and use

A

BITE between CD3 and CD19 which bridges T and ALL cells

relapsed/refractory ALL and MRD+ ALL after induction followed by transplant

148
Q

relapsed/refractory ALL treatments

A

blinatumomab (category 1)
inotuzumab ozogamicin
Brexucabtagene autoleucel (adult) tisagenlecleucel (AYA) or multi-agent chemo

then consider HCT

149
Q

maintenance regimens in ALL

A

PH+: TKI added, monthly vincristine/prednisone for 2-3 years, may include weekly methotrexate + daily 6MP

Ph-: weekly MTX + daily 6MP + monthly vincristine/pred
or blinatumomab alternating with POMP (6MP vincristine, mtx, pred)

150
Q

Risk factors and Cytogenetic risk groups in ALL

A

Generally high risk
- age >35
- WBC >30 in BALL, >100in TALL
- ETP-ALL in TALL
- see below for risk cytogenetics

good risk: hyperdiploidy (trisomies), t(12;21), t(1;19), DUX4

poor risk is:
- hypodiploidly
-p53
-KMT2A
- IgH, HLF, ZNF384, MEF2D rearranged
- BCR-ABL like with JAK-STAT, ABL rearrangements
- antecedent CML or t(9;22) with IKZF1
- iAMP21
- IKZF1
complex karyotype

151
Q

inotuzumab moa and use, risk to worry about

A

CD-22 antibody bound to calicheamicin (antibody-drug conjugate)

liver toxicity, increased risk of liver damage and SOS with transplant

152
Q

gemtuzumab ozogamicin moa and use

A

CD33 and calicheamicin ADL for AML CD33+ cells

153
Q

CMV donor mismatch

A

better if donor is CMV+ and recipient is CMV- as new marrow has antibodies against it due to prior exposure

154
Q

engraftment syndrome symptoms, timing, treatment

A

fever and skin rash within a week of transplantation, pulmonary infiltrates, edema, weight gain, liver/kidney dysfunction, encephalopathy. rule out infection, and treat with steroids

155
Q

late-phase transplant infections

A

impaired cellular immunity lead to infection risk to aspergillus, PJP, encapsulated bacteria and VZV

156
Q

which anti-rejection drug can cause a TMA and what testing show

A

cyclosporine can cause a drug-induced TMA, ADAMSTS13 is not very low like in TTP, stop the drug or reduce dose, +/- plasma exchange or infusion

156
Q

MRD for ALL sensitivity requirement

A

10^-4, 6-color flow cytometry
PCR/NGS is 10^-6

157
Q

T ALL relapsed refractory treatment

A

nelarabine +/- etop + cyclophos

158
Q

angioimmunoblastic T-cell lymphoma presentation and immunophenotype, treat

A

rash, fluid retention, lymphadenopathy, hypergammaglobulienmia, HSM, eosinophilia, hemolytic anemia

T cell lymphoma that is CD4+, CD8+ with perivascular infiltrates

BV +CHP vs CHOP

159
Q

loncastuximab tersirine-lpyl moa and use

A

CD19 antibody drug conjugate with alkylating agent for relapsed or refractory DLBCL

160
Q

CAR-T matching by disease site and target

Brexucabtagene
Axicabtagene
Tisagenlecleucel
Lisocabtagene
Idecabtagene
ciltacabtagene

A

Brexucabtagene- BALL, Mantle (BAD)
Axicabtagene- Follicular, Marginal and DLBCL/PMBCL
Tisagenlecleucel- (T)eens with BALL, follicular, DLBCL (3rd line)
Lisocabtagene- DLBCL, PMBCL
Idecabtagene- myeloma (4 or more lines)
ciltacabtagene- myeloma (4 or more lines)

CD19 for lymphomas, BCMA for myeloma car-t

161
Q

occular MALT lymphoma treament options and associated bug

A

chlamydia psittaci, treat with doxycycline which can cause regression

or radiation or surgery or rituximab

162
Q

mantle cell maintence after AST

A

rituximab +/- BTK with OS benefit

163
Q

romidepsin moa and use

A

HDAC- histone deacetylase inhibitor approved for yse in cutaneous T-cell lymphoma

164
Q

erdheim chester disease what is it and treatment

A

non-langerhans histiocytic disorder

infiltrative disease into bone and other tissues

with or without mapK mutation can be treated with cobimetinib

BRAF V600E MUTATIONS: vemurafenib

other: cytarabine or cladribine without mutation

165
Q

polatuzumab moa and use

A

ADC targeting CD79b on B cell receptor which is used in DLBCL

166
Q

tafasitamab-cxix moa and use

A

CD19 antibody given with lenalidomide in refractory DLBCL third line or later

167
Q

follicular lymphoma treatment lines

A

BR
R2
3rd line- copanlisib, tazemetostat, car-t or mosuntuzumab

168
Q

Mosuntuzumab moa and use

A

BITE between CD3 and CD20 approved in FL after 2 or more lines of therapy

169
Q

copanlisib moa and use and risk

A

PI3K inhibitor alpha and delta 3rd line for R/R follicular lymphoma, look out for elevated glucose

170
Q

High risk cytogenetics in amyloid and why

A

t(11;14) due to bortezomib resistance

treatment first line is dara-CyBorD

this is a good risk cytogenetic profile in myeloma however

171
Q

11;14 in myeloma sensitive to what

A

venetoclax which is given with dex with or without dara or PI

172
Q

cryoglobulins in myeloma or waldenstoms, symptoms and type

A

type I which are IgG or IgM

cause hyperviscosity and thrombosis such as raynauds, digital ischemia, livedo reticularis and neurologic symptoms

treat underlying disorder, avoid cold

173
Q

Transthyretin TTR amyloidosis treatment

A

progressive cardiomyopathy
liver transplant
tafamidis- binds TTR and reduces amyloid formation
Inotersen- oligonucleotide inhibitor of hepatic TTR production for neuropathy
patisiran- siRNA prevents TTR production by the liver, approved for neuropathy improvement

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