Myeloproliferatic Syndromes and AML

Question 1

myelodysplastic syndromes

Answer 1

clonal disorder from HSC lesion –>
dysfunctional cells and premature apoptosis
peripheral cytopenias despite hyper cellular bone marrow

Question 2

bone marrow biopsy in MDS

Answer 2

normo or hypocellular
increased myeloblasts
abnormal localization
increased iron (ringed sideroblasts, increased iron storage)

Question 3

types of MDS and #s of blasts in BM

Answer 3

20% AML

Question 4

IPSS scoring system

Answer 4

for MDS - higher score = bad

3 factors: 1. # blasts in marrow, 2. # cytopenias, 3. karyotype

Question 5

tx of MDS

Answer 5

supportive care
growth factors
immunomodulation
chemo w/ demethylating agents
high dose chemo (induction
allogeneic HSC transplant

Question 6

sx of AML

Answer 6

cytopenias

• neutropenia – infection
• anemia – fatigue, dyspnea
• thrombocytopenia - bleeding, bruising

Question 7

AML dx

Answer 7

> 20% bone marrow blasts!

cytochrome stains, flow cytometry

Question 8

BMB in AML

Answer 8

hypercellular
>20% blasts - prominent nucleoli, high nucleus-to-cytoplasm ratio, AUER RODS
cytochemical analysis: MPO+

Question 9

flow cytometry markers - B cell lineage AML

Answer 9

CD19, 20, 22

Question 10

flow cytometry markers - T cell lineage AML

Answer 10

CD3, 4

Question 11

flow cytometry markers - myeloid lineage AML

Answer 11

CD33

Question 12

favorable karyotypes in AML

Answer 12

Inv(16)
t(15;17)
t(8:21)

Question 13

bad karyotypes in AML

Answer 13

trisomy 8, monosomy 7, del 5q

Question 14

what drugs cause AML/MDS?

Answer 14

alkalating agents

topoisomerase type II

Question 15

MPO+

Answer 15

indicates AML

Question 16

tx for AML

Answer 16

induction - myeloablative, goal is remission
consolidation - 1-4 more courses of chemo after remission
allogeneic stem cell transplant

Question 17

acute promyelocytic leukemia

Answer 17

cytogenetics: t(15,17), PML/RAR-alpha molecualr sequence

coagulopathies, bleeding common –> DIC

Question 18

tx for APL

Answer 18

ATRA - encourages maturation and apoptosis of malignant blasts
Arsenic - degrades PML/RAR-alpha protein –> differentiation and apoptosis of APL blasts

Question 19

ATRA side effects

Answer 19

hyperleukocytosis –> all WBCs grow up at once

retinoic acid syndrome: fever, peripheral edema, resp distress - occurs in 25% pts, can be fatal