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Flashcards in AML Deck (29)
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1

t(8:21)

Runx1-Runx1T1

2

t(8:21)

--fusion protein of two transcription factors
--5% of AML cases
--Class II mutant (dominant repressor of myeloid maturation)

3

t(8:21)...Clinical presentation

younger pts/ kids

4

t(8:21)...Morphology

--Some maturation to myelocytes
--Occasional crystallization of granule contents (“Auer rods”)

5

t(8:21)...Immunophenotyping

CD34+
HLA-DR+
CD13+
CD33 weak

6

t(8:21)...Prognosis

Good Response to chemo

7

t(15;17)(q22;q12)

PML-RARA

8

t(15;17)

--Fusion of PML (transcription factor) with RARA (transcription factor - retinoic acid receptor)
--5-8% of AML cases

9

t(15;17)

Previously called AML-M3 or acute promyelocytic leukemia!!! (APL)

10

t(15;17)

--Dominant negative blockade of normal RARA
--Inhibits Granulocyte differentiation (Class II mutation)
--(requires concomitant Class I mutation)

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t(15;17)... Treatment (see Pharm form more on treating APL)

--PML-RARA itself can be blocked with an RA analogue (ALL TRANS RETINOIC ACID, or ATRA)
--ATRA induces differentiation of the blasts to granulocytes CLINICAL REMISSION
***OTHER TRANSLOCATIONS TO RARA FROM OTHER LOCATIONS THAT PRODUCE APL MAY NOT RESPOND TO ATRA!!!!

12

t(15;17)...Clinical Presentation

--DIC
--Severe thrombocytopenia

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t(15;17)...Morphology

--Big blasts
--Cleaved, "bat wing" nuclei
--Many cytoplasmic granules
--Aurer rods in stacks

14

t(15;17)...Immunophenotype

Weak/absent CD34
HLA-DR
CD13+
CD33+

15

t(15;17)...Prognosis

Good if diagnosis is made

16

CBFB-MYH11

inv(16)
OR
t(16:16)

17

CBFB-MYH11

--Fusion protein of transcription factors w/ MYH1
--5-8% of AML cases
--Class II Mutant (dominant negative repressor of myeloid maturation)
--Probably requires concurrent Class I mutation

18

CBFB-MYH11...Clinical presentation

younger pts/ kids

19

CBFB-MYH11...Morphology

--Mixed granulocyte-monocyte features ("Myelomonocytic")
--Increased eosinophils in blood and marrow

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CBFB-MYH11...Immunophenotype

CD34+
CD117+ (blasts)
CD13+
CD33+ (granulocytes)
CD14+
CD11b+ (monocytes)

21

CBFB-MYH11...Prognosis

Variably poor; optimal w/ high dose cytarabine
(SEE PHARM NOTES)
inv(16) prognosis good

22

AML w/ normal cytogenetics

40-50% of AML cases

23

AML w/ normal cytogenetics...Clinical Presentation

any age group

24

AML w/ normal cytogenetics...Morphology

undifferentiated OR
variably granulocytic OR
monocytic/monoblastic

25

AML w/ normal cytogenetics...Immunophenotype

Blast markers (CD34, CD117)+
can show any lineage markers

26

AML w/ normal cytogenetics...Prognosis

Depends on molecular genetics

27

AML w/ normal cytogenetics
NPMN1 or CEBPA+, FLT3-ITD-

60% 4 yr. survival
bone marrow transplant doesn't help

28

AML w/ normal cytogenetics
FLT3-ITD+ or NPM1-, CEBPA-, FLT3-ITD-

29% 4-yr survival
Bone marrow transplant helps

29

Poor prognosis AML

Complex (>3 clonal chromosomal abnormalities)
Partial monosomy (i.e. del(5q))
11q23
t(9:22)