Acute Myeloid Leukemia (Myeloid Neoplasms)

Question 1

AML occurs when mutations occur that impedes ______ (maturation/differentiation) of myeloid precursors

Answer 1

differentiation

Question 2

immature myeloid blasts accumulate in the bone marrow that causes ________ in the blood

Answer 2

cytopenia

Question 3

What is the median age for AML to occur?

Answer 3

60 years

Question 4

Classify AML

Answer 4

AML w/ genetic abberation
AML w/ MDS
AML asso. w/ therapy
AML not specific

Question 5

What are the mutations occuring in AML arising de novo or in young people?

Answer 5

t(8;21) - RUNX1/ETO fusion gene
inv(16) - CBFB/MYH1 fusion gene
t(15;17) - PML/RARA fusion gene

Question 6

What genetic changes occur in MDS related AML?

Answer 6

Deletions

Monosomies of 5 and 7

Question 7

What mutations are related with therapy related AML?

Answer 7

t(11q23;v) MLL FUSION GENES

Question 8

De novo cases of AML include mutation ________ {inv(16)/FLT3}

Answer 8

inv(16)

Question 9

inv(16) makes _______ fusion gene

Answer 9

CBFB/MYH1

Question 10

RUNX1/ETO fusion gene is made by translocation of ______

Answer 10

t(8;21)

Question 11

t(15;17) is responsible for ________ {MLL fusion genes/PML-RARA fusion gene}

Answer 11

PML-RARA fusion gene

Question 12

________ is responsible for MLL fusion genes

Answer 12

t(11q23;v)

Question 13

MLL fusion genes made by t(11q23;v) are _________ (MDS/therapy) related

Answer 13

therapy

Question 14

___________ {t(8;21)/t(9;22)} is associated with de novo AML that makes RUNX1-ETO fusion gene

Answer 14

t(8;21)

Question 15

CBFB/MYH1 is made by _______ {epigenetic changes/t(11q23;v)/inv(16)}

Answer 15

inv(16)

Question 16

Name the eight mutations that are associated w/ AML.

Answer 16

t(8;21)
inv(16)
t(15;17)
FLT3
t(11q23;v)
epigenetic changes
Cohesion complex
NPM mutation

Question 17

What do we find in the bone marrow of AML patients? (4M)

Answer 17

Myeloblasts
Monoblasts
Megakaryocytic differentiation
Marrow fibrosis
Rarely erythroid differentiation is seen

Question 18

What are the characteristics of myeloblasts?

Answer 18

Cytoplasm more than lymphoblasts
Auer rods
Azurophilic granules
Peroxidase positive

Question 19

What are the characteristics of monoblasts?

Answer 19

No auer rods

Non specific esterase positive

Question 20

What are the lab findings in AML?

Answer 20

Blast cells may be variable
they may be absent (aleukemic leukemia - do bone marrow biopsy to confirm)
may be less than 10,000
may be more than 100,000
Anemia
Neutropenia
Thrombocytopenia

Question 21

What are the clinical features of AML?

Answer 21

Fatigue
Recurrent infections
Mucocutaneous bleeding and petechiae

Question 22

What is the feature of monocytic variant of AML?

Answer 22

Monoblasts invade the skin (leukemia cutis) and gingiva

Question 23

Why does fatigue and infxn occurs in AML

Answer 23

Fatigue due to anemia

infxn due to neutropenia

Question 24

Why is there a bleeding tendency in AML?

Answer 24

low platelets

Leukemic cells release procoagulants and fibrinolytic factors

Question 25

AML can also present as soft tissue masses. Give some examples

Answer 25

myeloblastoma
granulocytic sarcoma
chloroma

Question 26

What is the prognosis of t(8;21) type of AML?

Answer 26

it is favourable

Question 27

____% cases of AML are favorable

Answer 27

60%

Question 28

t(15;17) has the best prognosis and is curable by ______ and _______ salts.

Answer 28

ATRA

arsenic salts

Question 29

mutations in ____________ suggest a worse prognosis

Answer 29

kinase FLT3

Question 30

Molecular changes in AML often include

______ overexpression

Answer 30

CD33

Question 31

Treatment with _________, helps overcome the block in cell differentiation caused by t(15;17) PML-RARA fusion gene

Answer 31

retinoic acid

Question 32

Cell death with release of the granules into

the peripheral blood can cause _______

Answer 32

disseminated intravascular coagulation