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HEM TEST 2024 > AML facts > Flashcards

AML facts Flashcards

1
Q

AML with myelodysplasia-related
gene mutations

A

ASXL1, BCOR, EZH2, RUNX1, SF3B1, SRSF2, STAG2, U2AF1, ZRSR2

BREASSSUZ- BCOR, RUNX1, EZH2, ASXL1, SRSF2, SF3B1, STAG2, U2AF1, ZRSR2

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2
Q

hierarchy of AML classification

A

Recurrent mutational abnormalities
# p53
# myelodysplasia-related gene mutation
# myelodysplasia-related cytogenetic abnormalities
# NOS

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3
Q

qualifiers for AML classification

A

Therapy related
Prior MDS, MDS/MPN
Germline disposition

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4
Q

AML with myelodysplasia-related cytogenetic abnormalities

A

Complex karyotype
5
7
12
17
20

7+5=12
12+5=17
5+5+5+5=20

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5
Q

Therapy related AML after alkylating agents/radiation

A

5-7 years latency
preceded by MDS
complex karyotype

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6
Q

Therapy related AML after topoisomerase Tx

A

1-3 years latency
Antecedent MDS is rare
MLL rearrangement

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7
Q

Important germline mutations in AML and there presetation

A

DDX41- older age of presentation, thalasemia like
ANKRD26, ETV6, and RUNX1- Platelet defects
GATA2- immunodeficiency
Shwachman Diamond syndrome- pancreatic insufficiency+ skeletal dysplasia
Fanconi anemia- facial dysmorphism, squamous cell carcinomas, liver tumors
Dyskeratosis congenita with pulmonary fibrosis, liver cirrhosis,
and vascular anomalies

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8
Q

Precurser markers in AML

A

CD34, CD117, HLA-DR

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9
Q

Myeloid markers in AML

A

Cytoplasmic MPO, CD33, CD13

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10
Q

Monocytic markers in AML

A

CD14, CD36, CD64, lysozyme
CD4, CD38,
CD11c

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11
Q

Megakaryocytic markers in AML

A

CD41 (glycoprotein IIb/IIIa), CD61
(glycoprotein IIIa), CD36

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12
Q

MPAL immuphenotype

A

MPO/monocytic+ CD3 or (CD19+CD79a/CD22/CD10)

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12
Q

Erythroid markers in AML

A

CD235a (glycophorin A), CD71,
CD36

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13
Q

Favorable risk AML

A

inv16 (core binding factor)
t8:21 (RUNX1)
NPM1
bZIP in frame CEPBA

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14
Q

Intermediate risk AML

A

FLT3
t(9:11) MLL:KMT2A
NOS

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15
Q

Poor risk AML

A

T(6:9) -Basophilia
inv3- EVI1
t(8:16)
t(9:22)
MDS related mutations/cytogentic abnormalities
p53

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16
Q

timing of response assesment in AML

A

2 cycles of intensive therapy
180 days of VV

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17
Q

CPX-351

A

Post MDS (Hx or cyto(genetic))
pts 60-75
less mucositis and TRM
better OS (18% vs 10%)

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18
Q

IDH1 i combination in ND AML

A

AGILE
HMA +- Ivosidenib
Differentiation syndrome 14%
24 months median OS

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19
Q

Principal of tranplant decision in AML (risk/benefit)

A

When relapse rates are >35-40%

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20
Q

MAC vs RIC in AML

A

BMT CTN 0901 trial
improved OS with MAC
Marked reduction in relapse
mainly if MRD positive before transplant

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21
Q

Number of chemo cycles prior to transplant in AML

A

no advantage for >2 cycles

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22
Q

Giltretinib maintenance post transplant in AML

A

Advantage only if MRD positive pre- or post-HCT

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23
Q

VV maintenance in post transplant AML

A

still in research
seems safe and effective
5 days pf AZA (35mg/m2) with 14 days ven 400 mg

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24
Q

CHIP mutations in AML

A

DTA
DNMT3A, TET2, ASXL1

25
Q

Sorafenib maintenance trial after allo

A

SORMAIN
limitation- no midostaurin in induction

26
Q

GATA2-related MDS

A

Monosomy 7
young age
Hx of immunodeficiency

27
Q

ELN classification in transplant ineligible AML pts

A

not relevant
favorable and intermediate risk groups have same prognosis
TP53 and RUNX1 mutation are especially poor prognosis compared to other adverse risk

28
Q

When to consider LP in CR1 of AML

A

Monocytic differentiation
MPAL
WBC >40 at diagnosis
Extramedullary disease
FLT3 mutations

29
Q

RUNX1 mutated AML

A

presents with thrombocytopenia

30
Q

inv16

A

CBFB-MEH11

31
Q

t(8:21)

A

RUNX1-RUNX1LT1

32
Q

cutoff of 20% for AML

A

BCR:ABL1
CBPA

33
Q

AML with defining genetic abnormalitis (not requiring blast threshold)

A

PML:RARA
RUNX1-RUNX1LT1
CBFB:MYH11
DEK:NUP214
RBM15:MRTFA
KMT2A
MECOM
NUP98
NPM1

34
Q

AML inv16 morphology

A

eosinophils

35
Q

Down syndrome associated AML

A

GATA1 mutated
seen only in Down syndrome

36
Q

APL thresholds for PLT/Cryo

A

PLT 30-50
Fib 150-200

37
Q

Tx of differentiation syndrome

A

Dexa 10 mf 2/d
for 3 days after dyspnea reolves

38
Q

Tx of APL in CR2

A

auto transplant

39
Q

Type I FLT3 inhibitors

A

Midostaurin
Giltretinib
(active and non-active FLT3, TKD+ITD)

40
Q

Type II FLT3 inhibitors

A

Quizartinib
Sorafinib
(active FLT3, ITD)

41
Q

Quizartinib in AML

A

QUANTUM FIRST
pts 18-75
OS advantage in pts <60

42
Q

MORPHO trial in AML

A

maintenance gilt after ASCT
negative trial (0.051)
but OS advantage in MRD+ pts

43
Q

Number of somatic mutations on de novo AML

A

13
5 of them driver mutations

44
Q

ATRX mutations im AML

A

Thalassemia like phenotype

45
Q

AML biology def

A

Clonal somatic mutations with proliferation and maturation arrest
Acquisition over time

46
Q

Calculator for CCUS risk

A

CHRS

47
Q

CEBPA mutation

A

In frame bZIP mutation
Germaine CEBPA 100% penetrance. Good prognosis

48
Q

DDX41 germline mutation

A

Older age
Good risk

49
Q

GO SE

A

Prolonged thrombocytopenia
VOD

50
Q

MRD progression

A

1 log increase

51
Q

Onureg

A

Oral azacitidine
For maintenance in AML NPM1
with MRD +
Approved only for poor risk AML not going to tranplant

52
Q

Management of MRD failure NPM1 AML

A

Preemptive therapy with
ASCT with or without bridging

53
Q

Prognostic groups based for VV pts

A

P53- low benefit
NRAS, FLT3- int benefit
all the rest- marked benefit

54
Q

Is single agent venetoclx effective after MRD - on VV

A

no
high degree (50%) of relapse
not salvageable with reinitiating of vidaza

55
Q

Daunorubicin dosage in induction

A

90 better than 45
60 as effective as 90

56
Q

AML presenting with extreme leukocytosis

A

FLT3
RAS

57
Q

AML with t(6;9) blast count threshold

A

10%
Usually prominent basophilia

58
Q

APL with other RARA rearrangement blast count threshold

A

10%

59
Q

Management of prolonged (>42 days) cytpenia in VV Tx of AML

A

Shorten venetoclax duration
Reduce AZA dosage
Increase inter-cycle interval
Administer GCSF during expected nadir

60
Q

Management of prolonged (>42 days) cytpenia in VV Tx of AML

A

Shorten venetoclax duration
Reduce AZA dosage
Increase inter-cycle interval
Administer GCSF during expected nadir

HEM TEST 2024 (32 decks)

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