100 AML Flashcards by Medicine Katorse

Most common acute leukemia in older patients

AML

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Mean age at diagnosis of AML

67 years

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

False. Most cases of AML are idiopathic

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Germline mutations associated with increased risk for developing myeloid neoplasm

CEBPA,
DDX41
RUNX1
ANKRD26
ETV6
GATA2

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

True

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

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

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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

Congenital neutropenia or Kostmann syndrome

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Leading cause of therapy associated AML?

Anticancer drugs

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

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

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

Ionizing radiation
Benzene
Chloramphenicol
Phenylbutazone

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

Ankylating agents; abberations in chromosome 5 and 7

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Required to esbtalish the diagnosis of AML

Marrow or blood blast count of more than 20%

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

PML- RARA fusion product

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Fusion products associated with core binding factor AML

Fusion products RUNX1- RUNX1T1 and CBFB-MYH11

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

PML

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

RARA gene

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

PML RARA

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Provide the most important independent prognostic information on AML

Chromosome finding

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AML with this genetic charteristic is associated with very good prpgnosis

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

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AML with this cytogenetic property is associated with very poor prognosis

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

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

True

Favorable outcome: miR-181a

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One of the most important risk factor for AML

Age. Advancing age poor prognosis

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

True

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Why is advanced age associated with poor prognosis?

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

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

Cytopenia

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

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

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

True

Define complete remission

``` 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 ```

First symptoms among AML patients

Fatigue

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

10%

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

Myeloid sarcoma

Common sites for myeloid sarcoma

``` Skin Lymph mode GIT Soft tissue Testis ```

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

True

What time of leukemia is hemorrhagic complications common?

Acute promyelocytic leukemia

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

Mklonocytic subtypes and those with 11q23 chromosomal abnormalities

Hematologic findings usually present at diagnosis

Anemia

Median Leukocyte count in patient with AML

Median presenting Leukocyte count is about 15,000/ uL | Only 20% have counts more than 100,000 u/L

Two phases in the treatment of AML

Induction | Post remission management

Common used regimen in induction

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

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

Cytarabine

Anthracycline used in AML

Daunorubicin | Idarubicin

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

True

Best relapse prevention strategy currently available for AML

Allogeneic HCT

True or false. Relapsed AML is typically resistant to chemotherapy

True

Favored strategy for AML in CR1

Allogeneic HCT

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

Intermediate dose cytarabine (1-1.5 g/m2) | Induction therapy dose for cytarabine is 60-90 mg/kg

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

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

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

Allogeneic HCT | Alternative: donor transplant if no HLA matched donor

Diagnosed. AML. Refractory/ Relapse. What next?

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

Target platelet count in AML? Target Hgb?

Platelet count more than 10,000/ uL | Hgb more 7 g/dL

Acceptable regimens for empiric treatment of infection in neutropenic AML

``` Imipenem-cilastin Meropenem Piperacillin tazobactam Cefepime Ceftazidime ```

Antifungal treatment for fever that persists for 4-7 days

Caspofungin Voriconazole Isavuconazoniun Liposomal amphothericin B

Predictive of response to salvage therapy

Length of first complete remission

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

True

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

True

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

Tretinoin (ATRA)

What is the complication of ATRA?

APL differentiation syndrome

When does APL syndrome occur and how does it manifest

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.

What is the pathophysiology of APL?

Adhesion of differentiated neoplastic cells to pulmonary vasculature endothelium

How is APL managed?

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

Standard of care for APL

ATRA + ATO (arsenic trioxide)

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

False. ATRA + ATO is superior and thus the standard

Signal associated with long term disease free survival

Absent PML RARA by RT PCR

Standard of post remission monitoring of APL

Sequential monitoring of PML RARA by RT PCR

Remains the preferred reinduction therapy for relapsed patient

ATO

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

Negative: Autologous HCT Positive: allogeneic HCT

Bears the retinoid acid receptor

t(15;17)

Associated with myeloid sarcoma

t(8;21)

Important in distinguishing AML from ALL and other subtypes of AML

BMA with phenotyping

``` 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. 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

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

Infection