100 AML Flashcards
Most common acute leukemia in older patients
AML
Mean age at diagnosis of AML
67 years
True or false. Most cases of AML are due to radiation exposure
False. Most cases of AML are idiopathic
Germline mutations associated with increased risk for developing myeloid neoplasm
CEBPA, DDX41 RUNX1 ANKRD26 ETV6 GATA2
True or false. Somatic cell chromosome aneuploidy such as Downs Syndrome with Trisomy 21 is associated with increased incidence of AML
True
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
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
Leading cause of therapy associated AML?
Anticancer drugs
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
What other exposures result in home marrow failure that may later involved into AML
Ionizing radiation
Benzene
Chloramphenicol
Phenylbutazone
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
Required to esbtalish the diagnosis of AML
Marrow or blood blast count of more than 20%
Diagnosis of Acute myeloid leukemia is based on the presents of this genetic findings
PML- RARA fusion product
Fusion products associated with core binding factor AML
Fusion products RUNX1- RUNX1T1 and CBFB-MYH11
Important in many cellular processes including cell growth control, apoptosis and senescene
PML
This gene encodes a number of nuclear hormone receptor family of transcription factors
RARA gene
This fusion gene suppresses gene transcription and block differentiation beyond promyelocyte stage
PML RARA
Provide the most important independent prognostic information on AML
Chromosome finding
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
AML with this cytogenetic property is associated with very poor prognosis
Complex karyotype
t(6;9)
Inv(3)
-7
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
One of the most important risk factor for AML
Age. Advancing age poor prognosis
True or false. 85% of APL can achieve long term survival if treated with ATRA and may not need bone marrow transplantation.
True
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
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