AML Flashcards

1
Q

Threshold concerning for leukostasis in AMl

A

50k

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2
Q

What is considered treatment related AML?

A

Exposure to prior chemo, radiation, or ***immunosuppressive drugs

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3
Q

Gene mutation associated with topoisomerase II inhibitors in AML

A

11q23 (II looks like an 11)

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4
Q

Timeframe for treatment related AmL from topoisomerase exposure

A

1-3 years post treatment
*No MDS phase

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5
Q

Drug classes associated with therapy related AML

A
  • anthracyclines (topoisomerase II)
  • alkylating agents (eg cytoxan)
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6
Q

Cytogenetic abnormalities associated with exposure to alkylating agents in AML

A

5 and 7 deletions (present 5 and 7 years after)

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7
Q

Timeframe for treatment related AML from alkylating agents

A

5-7 years
*preceeding MDS phase

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8
Q

Change in CEBPA classification for favorable risk category

A
  • not biallelic. It’s now any bzip in frame mutated CEBPA
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9
Q

Risk stratification of FLT3 in AML

A

All intermediate now regardless of NPM1 (improved outcomes from FLT3 targeted therapy)

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10
Q

Indications for LP for de novo AML

A

1) WBC>30K
2) monocytic or myelomonocytic
3) FLT3 mutant
AND symptomatic

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11
Q

Initial management of leukostasis

A
  • hydrea
  • Given myeloid lineage on flow, 1-2 g cytarabine (can also be used) vs. cytoxan
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12
Q

Allopurinol mechanism of action

A

inhibits xanthine oxidase

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13
Q

Rasburicase mechanism of action

A

catalyzes enzymatic oxidation of poorly soluble uric acid into an inactive and more soluble metabolite allantoin

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14
Q

What is core binding factor AML?

A

AML with inv(16) or t(8;21)

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15
Q

Standard of care for CBF AML

A

7+3 + gemtuzumab ozogamicin (dramatic improvement in outcomes)

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16
Q

1) gemtuzumab mechanism 2) SE’s to know with gemtuzumab

A
  • increased risk of VOD (sinusoidal obstruction syndrome)
  • myelosuppression
  • increased liver toxicity
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17
Q

generic name of vyxeos

A

liposomal-daunorubicin/cytarabine

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18
Q

Treatment options for frail patient that is IDH1 mutant

A

1) HMA + venetoclax
2) HMA + ivosidenib 500 mg daily for at least 2 years + HMA q28 days (Preferred -AGILE - OS 24 vs. 8 months)

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19
Q

High dose cytarabine (HiDAC) SE to know + management

A
  • cerebellar toxicity
  • permanent discontinuation
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20
Q

Prophylaxis required when giving HiDAC

A

Steroid eye drops

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21
Q

Blast cutoff for AML diagnosis

A

Greater than 20%

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22
Q

What is 7 + 3

A

7 days cytarabine
3 days anthracycline

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23
Q

IDH1 targeted drug

A

Ivosidenib

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24
Q

IDH2 targeted drug

A

Enasidenib

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25
Q

AML immunophenotype

A

CD13+, CD33+, MPO+, CD34+

26
Q

Morphologic CR in AML

A

<5% blasts

27
Q

Consolidation for patient requiring 2 inductions in AML in CR2

A

Allo-HSCT

28
Q

Options for relapsed/refractory AML - “remission reinduction”

A

1) salvage combination chemotherapy followed by allo-HSCT (Phase 2 – impressive response rates but very toxic)
2) HMA + venetoclax if not received upfront, then allo-HSCT
3) Clinical trial followed by allo-HSCT
4) Add targeted therapy if available:
Given IDH mutation, add IDH inhibitor to regimen, e.g. HMA/venetoclax
Given FLT3 ITD or TKD mutant (prior FLT3 doesn’t exclude), gilteritinib followed by allo-HSCT (OS benefit)
Given CD33 and not used previously, gemtuzumab followed by allo-HSCT

29
Q

Most commonly used systemic therapy regimens for re-induction

A

(FLAG-IDA + venetoclax)
MEC

30
Q

What is FLAG-IDA?

A

Fludarabine, cytarabine, plus G-CSF , idarubicin

31
Q

What is MEC?

A

M - mitoxantrone
E - etoposide
C - cytarabine

32
Q

Favorable risk AML mutations

A

1) t(8;21)(q22;q22.1)/RUNX1::RUNX1T1
2) inv(16)(p13.1q22) or t(16;16)(p13.1;q22)/CBFB::MYH11
3) Mutated NPM1 without FLT3-ITD
4) bZIP in-frame mutated CEBPA

33
Q

Other AML RF’s

A

Benzene

34
Q

Germline mutations associated with increased risk of AML

A

CEBPA
DDX41

35
Q

AML defining genetic abnormalities

A

*NPM1 + RUNX1:RUNX1 + CBFB:MYH11 + DEK::NUP214 + RBM15::MRTFA fusion + KMT2A + MECOM + NUP98 + CEBPA

36
Q

What is the answer in terms of genetic abnormality with question showing peripheral smear full of eosinophils?

A

AML with inversion 16

37
Q

Management of AML with inversion 16

A

gemtuzumab

38
Q

What is AML-MRC?

A

AML with myelodysplasia related changes?

39
Q

Board answer to management of Down syndrome patient with MPN + mutation

A
  • observation (erythromegakaryocytic MPN, it spontaneously resolves over the first few months of life)
  • GATA1
40
Q

Genetic abnormality for patient presenting with basophilia

A

t(6;9)

41
Q

Genetic abnormality associated with AML presenting with thrombocytosis

A

inv(3) or T(3;3)

42
Q

What does quizartinib target

A

FLT3-ITD
*not TKD

43
Q

Giltertinib approval

A

Relapsed/refractory, not induction

44
Q

Common mutation in therapy related AML

A

TP53 (30-40%)

45
Q

Management of p53 mutant AML

A

clinical trial (we have no good standard therapies)

46
Q

Standard for defining who is ineligible for induction chemotherapy in AML

A
  • Age >75
  • CHF requiring treatment or EF <50%
  • DLCO <65% or FEV1 <65%
  • ECOG 2-3, can be disease dependent
47
Q

Board answer for elderly patient unfit for induction chemo

A

HMA + venetoclax

48
Q

Venetoclax dosing w/ HMA + venetoclax

A
  • continuous is board answer
  • small but compelling abstract for 14 days so a lot of centers are using 14 day dosing
49
Q

New IDH inhibitor recently approved

A

olutasidenib (only approved in relapsed/refractory setting)

50
Q

Preferred conditioning and clinical benefit for patients MRD+ prior to transplant

A
  • myeloablative conditioning (OS benefit vs. RIC w/ genomic evidence of disease prior to transplant)
51
Q

Age as a RF for AML

A

Age over 65 is an independent predictor of poor prognosis

52
Q

Caveat to genetically defined AML

A

Has to be at least 10% blasts in marrow or blood

53
Q

Indication for germline testing in AML

A

Age <40 years

54
Q

Role for maintenance azacitidine

A

Approved for pts >55 in CR1 who aren’t candidates for intensive consolidation therapy

55
Q

t(6;9) risk stratification

A

adverse

56
Q

Induction therapy for CBF AML patients with CHF

A

Fludarabine, cytarabine, GCSF, and gemtuzumab

57
Q

What is the role of gemtuzumab in AML induction?

A
  • approved in addition to 7+3 in adults with intermediate or favorable (NOT adverse) but truth is results have been mixed from trials
58
Q

Basic physiology of NPM1 mutation

A

Abnormal nuclear export signal

59
Q

Clinical significance of -7

A

high risk

60
Q

Preferred conditioning regimens

A

IF significant comorbidities (DM1, cardiac), reduced intensity
IF fit, myeloablative

61
Q

Preferred reduced intensity conditioning regimen

A

Fludarabine, busulfan

62
Q

What are the myeloablative conditioning regimens

A
  • cyclophosphamide/busulfan
  • cyclophosphamide, TBI
  • Etoposide, TBI