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ABIM Hematology > Malignant Hematology > Flashcards

Malignant Hematology Flashcards

1
Q

Define fav risk AML?

A
  1. t(8;21) RUNX1-RUNX1T1
  2. inv(16) CBFB-MYH11
  3. NPM1mut with FLT3 wild-type or FLT3 low
  4. inframe bZIP mut in CEBPA (aka biallelic mut CEBPA)
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2
Q

Mutation that confers resistance to imatinib in patients with WM?

A

CXCR4

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3
Q

Reason to start ppx steroids to prevent differentiation syndrome in APL?

A

WBC > 10k

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4
Q

Regimens for high risk APL?

A

All regimens include a combination of THREE:

-all have ATRA
-then two of the following: ATO is preferred (avoid if prolonged QTC) along with either idarubicin/daunorubicin (avoid if low EF) or gemtuzumab

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5
Q

Treatment related AML with 11q23 rearrangement?

A

topoisomerase inhibitor, occur 1-3 years after treatment.

Irinotecan
Etoposide
Doxorubicin/Idarubicin

Essentially any -rubicin, -tecan, -poside

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6
Q

IHC for erythroid AML?

A

CD 235a

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7
Q

IHC for megakaryocytic AML?

A

CD41 and CD61. associated with trisomy 21

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8
Q

IHC for monocytic AML?

A

CD11c, CD14, and CD64

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9
Q

Intermediate risk AML cytogenetics?

A

FLT3 mut regardless of NPM1
t(9;11)

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10
Q

ANC and PLT goal for repeat BMB following induction for AML?

A

ANC> 1k and PLT >100k

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11
Q

Following induction for AML, repeat BMB needs what percentage of cellularity to be adequate?

A

> 20% (blasts need to be <5% to rule out residual diseasE)

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12
Q

Reasons to perform screening LP for AML patients?

A

-monocytic differentiation
-mixed phenotype acute leukemia
-WBC>40k
-extramedullary disease
-high risk APL
-FLT3 mutations

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13
Q

Elevated bilirubin, edema, and RUQ pain within 21 days of HSCT?

A

SOS (VOD). treatment is supportive but can give defibrotide if not improving which is FDA approved.

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14
Q

Preferred Myeloablative conditioning regimen prior to HSCT?

A

IV busulfan(>10g/kg) and cyclophosphamide
IV/PO busulfan (>10g/kg) and fludarabine

Other regimens with Busulfan <10mg/kg would be considered Reduced Intensity Conditioning (RIC)

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15
Q

Umbilical single cord-blood unit benefits over double cord-blood unit for transplatation?

A

improved PLT recovery, less GVHD, and OS favored singe cord-blood

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16
Q

Benefits of adding Omidubicel-onlv (stem cell manipulation) to umbilical cord blood transplantations?

A

improved neutrophil recovery time and less incidence of infection

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17
Q

Which pts benefit from using higher dose daunorubicin (90mg/m2) for AML induction?

A

Pts less than 65 yo with int- or fav-risk and one of the following muts:
-NPM1
-FLT3
-DNTM3A

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18
Q

Tx for steroid refractory chronic GVHD?

A

ruxolitinib (preferred), calcineurin inhibitors (cyclosporine/tacrolimus) belumosudil, ibrutinib

mild cGVHD - topical treatment
mod cGVHD - systemic prednisone
severe cGVHD - both pred and ruxolitinib

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19
Q

Unique side effects to gilteritinib?

A

PRES
prolonged QT
Pancreatitis
Differentiation syndrome

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20
Q

Tx for post-transplant CMV refractory to ganciclovir, valganciclovir, cidofovir, or foscarnet?

A

Maribavir

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21
Q

Primary manifestations of chronic GVHD?

A

sclerotic skin changes, dry oral mucosa, dry eyes, elevated bilirubin, ulcerations/sclerosis of GI tract

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22
Q

HLA-DR2 and HLA-DR15 are associated with which type of MDS? Tx?

A

hypoplastic; immunosuppressive therapy like cyclosporine/ATG

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23
Q

Mutation seen in MDS with ringed sideroblasts?

A

SF3B1

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24
Q

Mutations assoc with atypical CML?

A

SETBP1
CSF3R
ETNK1

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25
Q

Unexplained cytopenia(s) despite BMB?

A

ICUS (Idiopathic cytopenia of undetermined significance)

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26
Q

Neoplastic clonal mutation without WHO criteria for hematologic neoplasm and no cytopenia.

A

CHIP (Clonal Hematopoiesis of Indeterminant Potential)

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27
Q

CHIP but with cytopenia

A

CCUS (Clonal cytopenia of undetermined significance)

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28
Q

Good risk MDS cytogen?

A

-Y, 5q-, 20q-, normal

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29
Q

Poor risk MDS cytogen?

A

chromosome 7 abnormalities; >2 abnormalities that are not good risk

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30
Q

Tx for low/int risk MDS with EPO<500 and symptomatic anemia? To this type of patient, when would you add GCSF?

A

ESA;
If no 5q deletion and ring sideroblasts >15% or >5% with SF3B1 mut)

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31
Q

Specific gene with chromosome 7 that leads to myeloid neoplasms (AML, MDS) when del(7) occurs?

A

SAMD9

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32
Q

Abatacept MOA?

A

binds CD80 and CD86, blocking interaction with CD28 on T cells – approved for PPX against acute GVHD

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33
Q

Tx for JMML (juvenile myelomonocytic leuk)?

A

Allo HCT. Use azacitadine as 1L therapy as you prepare for transplant

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34
Q

10-day course of decitabine approved for AML or MDS with unfavorable cytogen and which poor-risk molecular abnormality?

A

TP53

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35
Q

Indications to initiate tx for CLL?

A
  1. Hb<10 or PLT<100
  2. splenomegaly >6cm
  3. LAD>10cm
  4. rapid progression of lymphocytosis
  5. autoimmune complications
  6. symptomatic extranodal involvement (skin, kidney, lung etc)
  7. B-symptoms
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36
Q

Tx for 17p deleted CLL?

A

BTK and BCL-2 inhibitors are better than chemoimmunotherapy

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37
Q

Arrange CLL mut from best to worst prognosis

A

13q del (best), normal cytogenetics, trisomy 12, 11q del, 17p del (worst)

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38
Q

1L tx for hairy cell leuk? 2L? 3L?

A

1L: cladribine +/- rituximab
or pentostatin

2L: if relapse >2 years, then give same initial purine analog (cladribine, pentostatin etc) but include rituximab if not included previously. if <2 year, then change the purine analog

initiating treatment similar to CLL criteria

3L+: vemurafenib (BRAF+), Peginterferon, ibrutinib, Moxetumomab

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39
Q

mutation in CLL that leads to ibrutinib resistance?

A

C481S

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40
Q

IHC blastic plasmacytoid dendritic cell neoplasm? Tx?

A

CD4+, CD56+, CD123+, TCL1+

Tx Tagraxofusp (targets CD123)

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41
Q

Contraindication for FCR in CLL?

A

history of autoimmune complication from CLL including AIHA

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42
Q

How many days should you hold ibrutinib prior to surgery?

A

minor sx: 3-7 days before and after

major sx: 7 days before and after

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43
Q

When should you repeat BMBx following 1L treatment of hairy cell leukemia with cladribine?

A

4 months after completing therapy

44
Q

Indications for iron chelation in patients with thalassemia who receive chronic transfusions.

A
  1. ferritin > 1000
  2. MRI liver with iron concentration >3mg Fe/g dry weight
  3. > 10u pRBC per year
45
Q

What is 20-2-20 in smoldering myeloma?

A

high risk defined as:
BM plasma cells >20%
M-protein > 2g
Serum free light chain ratio >20

46
Q

What is R-ISS staging system in MM?

A

Stage I: B2 microglobulin <3.5 and albumin >3.5, normal LDH, and standard risk cytogenetics and normal LDH

Stage II: not stage I or stage III

Stage III: B2 microglobulin >5.5 and EITHER high risk cytogenetics or serum LDH > ULN

47
Q

high risk cytogenetics in MM?

A

t(4;14)
t(14;16)
t(14;20)
del 17p (TP53 mut)
gain 1q21
MYC translocation

48
Q

Immunophenotype difference between MM and plasma cell leukemia?

A

Plasma cell leukemia are CD20+ and CD56-

49
Q

1L tx for Systemic Light Chain Amyloidosis?

A

Daratumumab and hyaluronidase, cyclophosphamide, bortezomib, and dexamethasone

50
Q

Lymphoplasmacytic lymphoma that secretes IgM?

A

WM

51
Q

Tx for solitary plasmacytoma?

A

RT 40-50Gy

51
Q

POEMS Syndrome is an osteosclerotic myeloma treated as myeloma that stands for what?

A

Polyneuropathy (major)
Organomegaly
Endocrinopathy
M-protein (Major)
Skin changes

52
Q

Tx options and doses for Accelerated Phase CML?

A

Imatinib 600 qd
Dasatinib 140 qd
Nilotinib 400 BID
Bosutinib 500 qd

53
Q

Tx options and doses for Chronic Phase CML?

A

Imatinib 400 qd
Dasatinib 100 qd **
Nilotinib 300 BID **
Bosutinib 400 qd **

** = (preferred for intermediate or high risk)

54
Q

TKI for following mutations:

  1. T315I
  2. T315A
A
  1. Ponatinib, Asciminib, Omacetaxine, allo transplant
  2. bosutinib, nilotinib
55
Q

best TKI for CML with CNS disease?

A

Dasatinib as it crosses the BBB

56
Q

Unique side effect of nilotinib?

A

prolonged QTc and subsequent sudden death

pancreatitis

57
Q

Unique side effect of bosutinib?

A

diarrhea

58
Q

Criteria to stop TKI?

A
  1. age>18
  2. Chronic phase CML only with no hx of blast or accel phase
  3. on approved TKI for 3 years
  4. stable molecular response (MR4, BCR-ABL<0.01%)
  5. Access to a PCR test with detection of at least MR 4.5 (<0.0032%)
  6. Monthly molecular monitoring for first 6 mo, q2m mo7-12, then q3mo thereafter
  7. Resumption of TKI within 4 weeks of loss of MMR
59
Q

Define loss of MMR in CML?

A

if BCR-ABL transcript increases above 0.1%

60
Q

Define complete (or deep) molecular response in CML?

A

MR4.5 (<0.0032%)

61
Q

Define major molecular response in CML?

A

MR4 (<0.01%)

62
Q

When do you lower ponatinib dose in CML treatment?

A

When BCR-ABL < 0.1% (MMR), decrease from 45mg to 15mg to try to prevent vascular events

63
Q

Tx for low risk MF and symptomatic?

A

ruxolitinib or peginterferon or hydroxyurea

64
Q

Tx for High risk MF?

A

allo HSCT

if not transplant eligible, then ruxolitinib (PLT need to be >50k), or fedratinib (check thiamine first as can cause Wernickes, PLTS need to be >50k, can be given if refractory to ruxolitinib), or pacritinib (can be used in pt with PLTS<50)

All are JAK inhibitors – if going to stop these therapies, should use a taper

65
Q

FIP1L1-PDGFRa ?

A

most common mutation seen in hypereosinophilia in 10% cases. need FISH or PCR to detect 4q12 mutation. loss of CHIC2 protein.

SENSITIVE TO IMATINIB 100mg daily

66
Q

PDGFRb ?

A

Seen in MDS/MPN with eosinophilia.
chromosome 5q33

IMATINIB 400MG DAILY

67
Q

FGFR1 rearranged neoplasms?

A

MPN (eosinophilia) with aggressive progression to AML in 1-2 years

tx with AML/ALL chemotherapy followed by transplant

Pemigatinib is FGFR1 inhibitor

68
Q

CSF3R mutation?

A

Think Chronic Neutrophilic Leukemia. affect the JAK-STAT pathway

Try to get to a transplant. can use ruxolitinib but not as effective

69
Q

Tx for mastocytosis WITHOUT KIT D816V mutation?

A

imatinib

70
Q

Tx for mastocytosis with KIT D816V mutation?

A

Used as first line tx in advanced systemic mastocytosis:
Avapritinib (preferred)
midostaurin

Other options include:
cladribine
Peginterferon alpha

71
Q

Define hypereosinophilic syndromes?

A

eosinophil count >1500 for 6 months

72
Q

Which mutation to think about when you have anemia or MDS with ringed sideroblasts?

A

SF3B1

73
Q

Low risk ET on ASA with progressive splenomegaly with assoc pain?

A

start cytoreductive therapy with either hydrea, interferon or anagrelide

74
Q

DIPSS for MF?

A

1pt - age > 65
1pt - B symptoms
2pts - Hb<10
1pt- WBC>25k
1pt - blasts > 1%

High risk = 5pts (treat with transplant)

Low risk = if asymptomatic, observe. if symptoms, can give ruxolitinib, fedratinib, IFN, or hydrea

75
Q

Clinical features of PV but without JAK mutation, and with a family history of suggestive of AD inheritance?

A

EPOR mutation (Primary familial congenital polycythemia)

76
Q

Mepolizumab?

A

Can be used in hypereosinophilic syndrome (HES) where there is no actionable mutation found.

targets IL-5

77
Q

lenolidomide dosing for a patient on dialysis?

A

5mg daily. administer medication after dialysis on dialysis days

78
Q

Zometa is contraindicated at which CrCl?

A

<30

79
Q

Treatment for HIV/HHV8 negative Castleman’s disease (angiofollicular lymph node hyperplasia)?

A

Siltuximab (Ab against IL6)

80
Q

How does t(3;14) alter prognosis in DLBCL?

A

improved prognosis (it is also seen in FL)

81
Q

How does t(14;18) alter prognosis in DLBCL?

A

worse prognosis (main translocation seen in FL)

82
Q

how long is brentuximab maintenance following auto transplant in HL?

A

1 year

83
Q

Over 90% of HIV associated hodgkin lymphoma are associated with which virus?

A

EBV

84
Q

Good risk AML mutation with AD inheritance?

A

CEBPA

85
Q

RR AML with IDH1 mutation

A

Ivosidenib or Olutasidenib

86
Q

t8;14
t2;8
t8;22

A

Burkitt’s lymphoma
all lead to c-myc activation

87
Q

2L for mantle cell lymphoma?

A

BTK inhibitors (Acalabrutinib or zanubrutinib) or R-squared

88
Q

3L for mantle cell lymphoma?

A

brexucabtagene autleucel

89
Q

1L FL stage I,II?

A

RT +/- rituximab

IF noncontinguous disease, then rituximab +/- RT

90
Q

1L FL stage III, IV? (2L is the same options)

A

BR or BO
RCHOP or OCHOP
RCVP or OCVP
R2
(This is very similar to 1L tx of Marginal Zone Lymphoma but without Obinutuzumab as an option)

Give ritux alone in elderly.

Can give ritux or obinutuzumab as maintenance

91
Q

3L FL?

A

Copanlisib, Idelalisib, Duveisib
Tazemetostat (regardless of EZH2 status)

CAR-T with Axi or Tisa
T-cell engager (CD3/CD20) with Mosunetuzumab

92
Q

1L tx for Mantle Cell all have which chemo?

A

cytarabine

93
Q

Targets for the following PI3K inhibitors?
1. Idelalisib
2. Copanlisib
3. Duvelisib

A
  1. delta
  2. alpha and delta
  3. gamma and delta
94
Q

HD MTX dose?

A

> 3g

95
Q

poor risk cytogen in AL Amyloidosis?

A

t 11;14

96
Q

treatment for transthyretin mutation+ amyloid?

A

liver transplant

97
Q

In MM, treatment in R/R setting if t(11;14) present?

A

Venetoclax (BCL2 inhibitor)

98
Q

Which MGUS needs BMBx?

A

Intermediate or High risk MGUS, therefore if any one of the following then should get BMB:
IgA or IgM,
abrnomal FLC ratio
M protein >1.5

99
Q

lenolidomide maint dose in MM?

A

10mg daily. start ~100 days after transplant. minimum of 2 years of maint. continue indefinitely

100
Q

5th line option for MM?

A

Selinexor (XPO1 inhibitor) with dex

101
Q

Tx for pediatric-type FL (negative for t(14;18)?

A

resect if localized. otherwise can do RT or RCHOP

102
Q

Tx of ICANS neurotoxicity from CAR-T?

A

steroids
edema management with mannitol or hypertonic saline
seizure ppx

DO NOT GIVE TOCILIZUMAB IF THERE IS NO CRS (CYTOKINE RELEASE SYNDROME)

103
Q

BTK inhibitor effective against C481S mutation?

A

Pirtobrutinib - approved in R/R Mantle cell lymphoma after 2 lines of therapy

104
Q

low risk CP CML starting doses?

A

imatinib 400 daily
bosutinib 400 daily
dasatinib 100 daily
nilotinib 300 BID

105
Q
A

ABIM Hematology (4 decks)

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