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Hematology > 100 AML > Flashcards

100 AML Flashcards

1
Q

Most common acute leukemia in older patients

A

AML

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2
Q

Mean age at diagnosis of AML

A

67 years

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3
Q

True or false. Most cases of AML are due to radiation exposure

A

False. Most cases of AML are idiopathic

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4
Q

Germline mutations associated with increased risk for developing myeloid neoplasm

A 
CEBPA,
DDX41
RUNX1
ANKRD26
ETV6
GATA2
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5
Q

True or false. Somatic cell chromosome aneuploidy such as Downs Syndrome with Trisomy 21 is associated with increased incidence of AML

A

True

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6
Q

Down syndrome associated AML is typically what subtype and is associated with mutation in what gene?

A

Acute megakaryocytic subtype and is associated with mutation in the GATA1 gene

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7
Q

Another disorder that may evolve into AML that is due to mutations in the genes encoding the granulocyte colony stimulating factor receptor and neutrophil elastase

A

Congenital neutropenia or Kostmann syndrome

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8
Q

Leading cause of therapy associated AML?

A

Anticancer drugs

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9
Q

This anticancer agent is associated AML occurs 1-3 year after exposure. What genetic abberations are noted?

A

Topoisomerase II inhibitors associated with leukemia 1-3 years after exposure and shows abberations in chromosome 11q23

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10
Q

What other exposures result in home marrow failure that may later involved into AML

A

Ionizing radiation
Benzene
Chloramphenicol
Phenylbutazone

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11
Q

This agent is associated with AML 4-6 years after exposure and what is the associated with aberrations

A

Ankylating agents; abberations in chromosome 5 and 7

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12
Q

Required to esbtalish the diagnosis of AML

A

Marrow or blood blast count of more than 20%

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13
Q

Diagnosis of Acute myeloid leukemia is based on the presents of this genetic findings

A

PML- RARA fusion product

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14
Q

Fusion products associated with core binding factor AML

A

Fusion products RUNX1- RUNX1T1 and CBFB-MYH11

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15
Q

Important in many cellular processes including cell growth control, apoptosis and senescene

A

PML

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16
Q

This gene encodes a number of nuclear hormone receptor family of transcription factors

A

RARA gene

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17
Q

This fusion gene suppresses gene transcription and block differentiation beyond promyelocyte stage

A

PML RARA

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18
Q

Provide the most important independent prognostic information on AML

A

Chromosome finding

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19
Q

AML with this genetic charteristic is associated with very good prpgnosis

A

t(15;17) - - 85% cured
t(8;21) - - 55% cured
Inv(16)
No cytogenetic abnormality - - 40% cured

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20
Q

AML with this cytogenetic property is associated with very poor prognosis

A

Complex karyotype
t(6;9)
Inv(3)
-7

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21
Q

True or false. Overexpresion of miR-155 and miR-3151 predicts unfavorable outcomes in AML. Which overexpression predicts favorable outcome?

A

True

Favorable outcome: miR-181a

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22
Q

One of the most important risk factor for AML

A

Age. Advancing age poor prognosis

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23
Q

True or false. 85% of APL can achieve long term survival if treated with ATRA and may not need bone marrow transplantation.

A

True

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24
Q

Why is advanced age associated with poor prognosis?

A
  1. Influence on the ability to survive induction therapy due to coexisting medical comorbidities
  2. Each successive decade of age, a greater portion have intrinsically more resistant disease
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25
Q

Clinical feature associated with lower complete remission rate and shorter survival time

A

Cytopenia

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26
Q

True or false. Complete Remission is higher in patients who had anemia, leukopenia, and thrombocytopenia for more than 3 months before diagnosis of AML

A

False. Cytopenia is associated with lower complete remission in patients with AML

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27
Q

True or false. Among patients with hyperleukocytosis (more than 100,000/ uL), early CNS bleeding and pulmonary leukostasis contribute to poor outcomes

A

True

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28
Q

Define complete remission

A 
On peripheral blood
Neutrophil count more than 1,000
Platelet count more than 1,000
Absent circulating blasts
On bone marrow
Less than 5% blasts
Absent Auer rods
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29
Q

First symptoms among AML patients

30
Q

Fever with or without identifiable cause is the is the initial presentation in patients for how many percent

31
Q

Tumor mass consisting of myeloid blasts occurring at anatomic sites other than bone marrow

A

Myeloid sarcoma

32
Q

Common sites for myeloid sarcoma

A 
Skin
Lymph mode
GIT
Soft tissue
Testis
33
Q

True or false. Patients with myeloid sarcoma is associated with chromosome aberrations

34
Q

What time of leukemia is hemorrhagic complications common?

A

Acute promyelocytic leukemia

35
Q

Infiltration of the gingiva, skin, soft tissues, or meninges with leukemic blasts is characteristics of what?

A

Mklonocytic subtypes and those with 11q23 chromosomal abnormalities

36
Q

Hematologic findings usually present at diagnosis

37
Q

Median Leukocyte count in patient with AML

A

Median presenting Leukocyte count is about 15,000/ uL

Only 20% have counts more than 100,000 u/L

38
Q

Two phases in the treatment of AML

A

Induction

Post remission management

39
Q

Common used regimen in induction

A

Cytarabine + anthracycline 7+3

Cytarabine for 7 days and anthracycline day 1, 2 and 3

40
Q

Cell cycle S phase specific antimetabolite that becomes phosphorylated intracellularly to an active triphosphate firm that interferes with DNA synthesis

A

Cytarabine

41
Q

Anthracycline used in AML

A

Daunorubicin

Idarubicin

42
Q

True or false. Post remission therapy is designed to eradicate residual leukemic cells to prevent relapse and prolong survival

43
Q

Best relapse prevention strategy currently available for AML

A

Allogeneic HCT

44
Q

True or false. Relapsed AML is typically resistant to chemotherapy

45
Q

Favored strategy for AML in CR1

A

Allogeneic HCT

46
Q

Diagnosed. AML. Favorable risk . Induction therapy done. If complete remission,

A

Intermediate dose cytarabine (1-1.5 g/m2)

Induction therapy dose for cytarabine is 60-90 mg/kg

47
Q

Diagnosed. AML. Intermediate risk . Induction therapy done. If complete remission,

A

Allogeneic HCT–preferres
Autologous Hct if less than 60 yrs old
Intermediate dose cytarabine

48
Q

Diagnosed. AML. Adverse risk . Induction therapy done. If complete remission,

A

Allogeneic HCT

Alternative: donor transplant if no HLA matched donor

49
Q

Diagnosed. AML. Refractory/ Relapse. What next?

A

Salvage therapy
Yes suitable donor: allogeneic HCT
No suitable donor: autologous HCT

50
Q

Target platelet count in AML? Target Hgb?

A

Platelet count more than 10,000/ uL

Hgb more 7 g/dL

51
Q

Acceptable regimens for empiric treatment of infection in neutropenic AML

A 
Imipenem-cilastin
Meropenem
Piperacillin tazobactam
Cefepime
Ceftazidime
52
Q

Antifungal treatment for fever that persists for 4-7 days

A

Caspofungin
Voriconazole
Isavuconazoniun
Liposomal amphothericin B

53
Q

Predictive of response to salvage therapy

A

Length of first complete remission

54
Q

True or false. Patient with complete remission more than 12 months have a higher change of attaining CR2.

55
Q

True or false. APL is highly curable and 85% achieve long term survival with current approaches

56
Q

Oral drug that induces differentiation of leukemic cells bearing t(15;17) where disruption of RARA gene encoding a retinoid acid receptor occurs

A

Tretinoin (ATRA)

57
Q

What is the complication of ATRA?

A

APL differentiation syndrome

58
Q

When does APL syndrome occur and how does it manifest

A

APL syndrome occurs during the first 3 weeks of treatment and characterized by fever, fluid retention, dyspnea, chest pain, pulmonary infiltrates, pericarditis effusion and hypoxemia.

59
Q

What is the pathophysiology of APL?

A

Adhesion of differentiated neoplastic cells to pulmonary vasculature endothelium

60
Q

How is APL managed?

A

Glucocorticoids
Chemotherapy
Support measures
Discontinuation of ATRA in severe cases of APL

61
Q

Standard of care for APL

A

ATRA + ATO (arsenic trioxide)

62
Q

True or false. ATRA + ATO is Inferior to ATRA+ idarubicin

A

False. ATRA + ATO is superior and thus the standard

63
Q

Signal associated with long term disease free survival

A

Absent PML RARA by RT PCR

64
Q

Standard of post remission monitoring of APL

A

Sequential monitoring of PML RARA by RT PCR

65
Q

Remains the preferred reinduction therapy for relapsed patient

66
Q

What the next step in CR2 in patients with RT PCR negative status? To those who remain positive on RT PCR

A

Negative: Autologous HCT
Positive: allogeneic HCT

67
Q

Bears the retinoid acid receptor

68
Q

Associated with myeloid sarcoma

69
Q

Important in distinguishing AML from ALL and other subtypes of AML

A

BMA with phenotyping

70
Q 
Character of complete remission in AML except
A. Platelet count more than 100,000
B. Neutrophil count 1000
C. Blast less than 5%
D. Hemoglobin more than 100 g/L
A

A. Platelet count more than 100,000
B. Neutrophil count 1000
C. Blast less than 5%
D. Hemoglobin more than 100 g/L–> Hemoglobin is not considered in determining complete remission

71
Q

Remain the major cause of Morbidity and death during induction post remission therapy in AML

Hematology (11 decks)

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