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Malignant Hematology > AML > Flashcards

AML Flashcards

1
Q

What 4 genetic abnormalities classify AML as favorable risk?

A

t(8;21)
inv(16)
Mutated NPM1 without FLT3-ITD
bZIP in-frame mutated CEBPA

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2
Q

What three genetic abnormalities classify someones AML as intermediate risk?

A

Mutated NPM1 with FLT3-ITD
Wild-type NPM1 with FLT3-ITD
t(9;11)

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3
Q

What 10 genetic abnormalities classify AML as adverse risk?

A

t(6;9)
t(v;11q23.3) (KMT2A-rearranged)
t(9;22)
t(8;16)
inv(3) or t(3;3)
t(3q26.2) MECOM(EV11)
del5q or -7
Complex karyotype, monosomal karyotype
Mutated TP53
Mutated ASXL1, BCOR, EZH2, RUNX1, SF3B1, SRSF2, STAG2, U2AF1or ZRSR2

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4
Q

How to risk stratify in APL? (low, intermediate, high)

A

Low: WBC <10, Platelet >40
Intermediate: WBC <10, Platelet <40
High: WBC >10

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5
Q

Most common known RF for development of AML?

A

Previous RT or chemo (particularly topo II inhibitors or alkylating agents)

less common: benzene, or familial syndromes

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6
Q

At what level of leukemic blasts put you at higher risk of leukostasis?

A

50k

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7
Q

The diagnostic criteria of requiring 20% blasts for AML is not required in what setting?

A

Defining cytogenetic abnormalities:
Complex karyotype
5q deletion
Monosomy 7 or 7q deletion
11q deletion
12p deletion
Monosomy 13
17p deletion
Isochromosome 17q
Defining somatic mutations: AXL1, BCOR, EZH2, SF3B1, STAG2, USAF1

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8
Q

What specific type of AML should you think of when told about someone with an AML picture and lots of eosinophils?

A

AML with inversion 16

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9
Q

AML associated with Down syndrome almost universally has what mutation?

A

GATA1

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10
Q

Acute Management of new diagnosis of APL

A

Treat coagulopathy with cryo to keep fibrinogen >150
Transfuse platelets to keep >30
Start ATRA STAT

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11
Q

What is the standard of care for low/intermediate risk APL?

A

ATRA + Arsenic

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12
Q

What is the standard of care treatment for high risk APL induction?

A

ATRA + Arsenic + Gemtuzumab
(Or ATRA + Arsenic + Idarubicin)

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13
Q

After starting a patient with APL on induction chemotherapy, on D7 they develop dyspnea, fever, peripheral edema, hypotension, and weight gain. What is the diagnosis?

A

Differentiation syndrome

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14
Q

How do you manage differentiation syndrome?

A

Steroids (Dex 10 mg BID)
Hold ATRA if renal/pulmonary failure

-For high risk APL, can consider prophylactic dexamethasone but that’s controversial

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15
Q

How do you monitor response to induction chemotherapy in APL?

A

RT-PCR for t(15;17)

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16
Q

What to do when, after completing induction chemotherapy for APL, you have a newly positive t(15;17) test?

A

Repeat test on Bone marrow biopsy in 2 weeks

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17
Q

Treatment for first relapse in APL (2 options)

A

Chemotherapy + ATRA + Arsenic
Gemtuzumab alone also an option

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18
Q

You have a patient with APL treated with ATRA+Arsenic and obtains CR1. 6 months later they relapse and you are able to achieve CR2 with chemotherapy + ATRA. What is the next treatment?

A

Autologous SCT

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19
Q

What is the appropriate dose of daunorubicin for induction chemotherapy for AML?

A

60 mg/m2 for most patients
Use 90 mg/m2 for younger, fitter patients

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20
Q

The addition of gemtuzumab for induction chemotherapy for AML is msot beneficial to which patients?

A

Favorable risk AML
Most data in core binding factor AML (inv(16) and t(8;21))

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21
Q

Induction chemotherapy for AML with FLT3-ITD mutation?

A

7+3+Midostaurin
Only if patient is under 60

22
Q

What is the mechanism of Quizartinib?

A

FLT-3 ITD mutation inhibitor.
**Will not work for FLT3-TKD

23
Q

What is the indication for Gilteritinib?

A

Relapsed/refractory AML FLT3-mutated AML

24
Q

Standard induction chemotherapy for fit patients with sAML or tAML?

A

CPX-351

25
Q

What are four clinical criteria to deem a patient elderly/not-fit for intensive induction chemotherapy?

A

Age >75
CHF requiring treatment or EF <50%
DLCO <65% or FEV1 <65%
ECOG 2-3

26
Q

Standard induction chemotherapy for unfit patients with AML

A

Azacitidine + Venetoclax

27
Q

Mechanism of action of Venetoclax

A

BCL-2 inhibitor.
BCL-2 stabilizes mitochondria and prevents activation of proapoptotic proteins. So, inhibiting BCL-2 enables apoptosis

28
Q

Mechanism of action of Ivosidenib

A

Inhibits IDH1

29
Q

3 Indications of Ivosidenib

A

R/R AML with IDH1 mutation
1st line monotherapy for IDH1 mutated AML for frail individuals
1st line in combination with azacitidine

30
Q

What is the standard post-remission consolidation treatment for favorable risk AML?

A

HiDAC
If CD33+, can get gemtuzumab

31
Q

You have a patient with AML in CR2, what is the best next step?

A

AlloHCT.

32
Q

When would you consider an alloHCT for a patient with favorable risk AML?

A

when they cannot tolerate/complete consolidation chemotherapy, or are MRD+

33
Q

How do you manage cerebellar toxicity due to cytarabine?

A

Stop cytarabine and don’t rechallenge

34
Q

A patient goes through induction chemotherapy with 7+3 + Gemtuzumab. They can’t tolerate HiDAC consolidation and so are going through transplant. What AE are they at higher risk for?

A

SOS/VOD

35
Q

Risk stratify according to ELN criteria for AML: t(6;9)

A

Poor

36
Q

Risk stratify according to ELN criteria for AML: t(v;11q23.3)/KMT2a rearranged

A

poor

37
Q

Risk stratify according to ELN criteria for AML: t(9;22)

A

poor

38
Q

Risk stratify according to ELN criteria for AML: t(8;16)

A

poor

39
Q

Risk stratify according to ELN criteria for AML: inv(3) or t(3;3)

A

poor

40
Q

Risk stratify according to ELN criteria for AML: -5 or del5q

A

poor

41
Q

Risk stratify according to ELN criteria for AML: monosomy 7

A

poor

42
Q

Risk stratify according to ELN criteria for AML: Mutated NPM1 without FLT3-ITD

A

favorable

43
Q

Risk stratify according to ELN criteria for AML: inv(16)

A

favorable

44
Q

Risk stratify according to ELN criteria for AML: t(8;21)

A

favorable

45
Q

Risk stratify according to ELN criteria for AML: bZIP in-frame mutated CEBPA

A

favorable

46
Q

Risk stratify according to ELN criteria for AML: Mutated NPM1 and FLT3-ITD

A

Intermediate

47
Q

Risk stratify according to ELN criteria for AML: wild-type NPM1 and FLT3-ITD

A

intermediate

48
Q

Risk stratify according to ELN criteria for AML: t(9;11)

A

Intermediate

49
Q

Favorable risk AML patient undergoes induction with 7+3+GO. D14 BMBx shows 13% cellularity and 2% blasts. What to do now?

A

Wait for count recovery and repeat marrow. If increased blasts are still seen, then salvage therapy/transplant search needs to start

50
Q

Which patients should be considered high risk for CNS disease? (6)

A

Monocytic differentiated
mixed phenotype
High WBC (>40K) at diagnosis
Extramedullary disease
high risk APL
FLT3 mutations

Malignant Hematology (13 decks)

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