Chapter 100 AML

Question 1

Most common acute leukemia in older patients

Median age 67 years

Answer 1

AML

Question 2

Subtype and gene involvement in down syndrome associated AML

Answer 2

Megakaryocytic subtype

GATA1 gene

Question 3

Germline mutations associated with increased risk of developing myeloid neoplasm

Answer 3

CEBPA
DDX41
RUNX1
ANKRD26
ETV6
GATA2

Question 4

Congenital neutropenia

Due to mutations in the gene encoding the gcsf receptor and neutrophil elastase

May evolve to AML

Answer 4

Kostmann syndrome

Question 5

Leading cause of therapy-associated AML

Answer 5

Anticancer drugs

Question 6

Alkylating agent associated leukemias
When?
Features?

Answer 6

4-6 years after exposure

Multilineage dysplasia and monosomy/aberrations im chromosomes 5 and 7

Question 7

Topoisomerase II inhibitor associated leukemias
When?
Features?

Answer 7

1-3 years after exposure

Monocytic features and aberrations involving chromosome 11q23

Question 8

Marrow (or blood) blast count of \_\_\_\_\_ is required to establish the diagnosis of AML except for AML with the recurrent genetic abnormalities:
1\_\_\_\_\_
2\_\_\_\_\_
3\_\_\_\_\_
4\_\_\_\_\_\_

Answer 8

More than or equal to 20%

t(15;17)
t(8;21)
inv(16)
t(16;16)

Question 9

Acute promyelocytic leukemia (APL)

Favorable clinical outcomes when properly treated

Answer 9

t(15;17)(q22;q12)

PML-RARA fusion product

Question 10

Core binding factor (CBF) AML

Favorable clinical outcomes when properly treated

Answer 10

t(8;21)(q22;q22)
inv(16)(p13.1q22)
t(16;16)(p13.1;q22)

RUNX1-RUNX1T1 and
CBFB-MYH11

Question 11

Only one cytogenetic abnormality invariably associated with specific morphological features

Answer 11

t(15;17)(q22;q12) with APL

Question 12

Several chromosomal abnormalities often associate primarily with one morphologic/immunophenotypic group

inv(16)(p13.1q22)

Answer 12

AML with abnormal bone marrow eosinophils

Question 13

Several chromosomal abnormalities often associate primarily with one morphologic/immunophenotypic group

t(8;21)(q22;q22)

Answer 13

Slender Auer rods
Expression of CD19
Increased normal eosinophils

Question 14

Several chromosomal abnormalities often associate primarily with one morphologic/immunophenotypic group

t(9;11)(p22;q23) and other translocations involving 11q23

Answer 14

Monocytic features

Question 15

Younger age

Answer 15

t(8;21)

t(15;17)

Question 16

Older age

Answer 16

del(5q)

del(7q)

Question 17

Myeloid sarcomas

Answer 17

t(8;21)

Question 18

DIC

Answer 18

t(15;17)

Question 19

Extramedullary sites of involvement at presentation, especially gingival hypertrophy

Answer 19

11q23 aberrations and monocytic leukemia

Question 20

DEK-NUP214

Answer 20

t(6;9)(p23;q34)

Question 21

MLLT3-KMT2A

Answer 21

t(9;11)

Question 22

FLT3-ITD
(fms-related tyrosine kinase 3, internal tandem duplication)

The more common of the FLT3 Mutations
Occurs in cytogenically normal AML (CN-AML)

Importance of identifying?

Answer 22

Useful as a prognosticator

May predict response to specific tx such as tyrosine kinase inhibitor (TKI)

Midostaurin
Quizartinib
Gilteritinib
Crenolanib
Sorafenib

Question 23

AML with minimal differentiation

Flow cytometry

Answer 23

Myeloid-specific antigens cluster designation (CD) 13 and/or 117

Question 24

Acute megakaryoblastic leukemia

Answer 24

Platelet-specific antigens CD41 and/or CD61

Question 25

Most important independent prognostic information

Answer 25

Chromosome findings at diagnosis

Question 26

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

monosomy 7, trisomy 8, 11q23 rearrangement, inv(16), trisomy 4, t(8;21)

Answer 26

Myeloid sarcoma

Question 27

Hemorrhagic complications are most commonly and classically found in ______

Answer 27

APL