Myelodysplastic Syndromes & AML

Question 0

peripheral lab findings in MDS

Answer 0

cytopenias
myeloid: left-shift
erythrocytes- macrocytosis
platelets- large, hypogranular
monocytosis

Question 1

Myelodysplastic Syndrome

Answer 1

mutated cell that divide but do not mature normally
results in
-dysfunctional cells and premature apoptosis
-peripheral cytopenias despite hypercellular bone marrow

Question 2

bone marrow in MDS

Answer 2

normo/hypercellular (ineffective hematopoiesis)
increase myeloblasts
dyspoiesis in 1 or more cell lines
abnormal localization
increased iron (sideroblasts)

Question 3

abnormal localization in bone marrow

Answer 3

• myeloid precursors away from bony trabeculae
• erythroid megakaryocytes away from sinusoids
• clustering of megakaryocytes

Question 4

Myelodysplastic Syndromes are NOT

Answer 4

curable

Question 5

suspected causes for primary MDS

Answer 5

cigarrete smoke
toxic chemicals
radiation

Question 6

suspected causes for secondary/treatment-related MDS

Answer 6

younger age of onset
cancer treatment modalities
chromosomal abnormalities
poor prognosis

Question 7

International Prognostic Scoring System ranges from

Answer 7

0-3.5

Question 8

prognosis depends on 3 factors

Answer 8

number of blasts in marrow
number of cytopenias
karotype

Question 9

treatment MDS

Answer 9

supportive care
Growth factors
immunomodulatory agents
chemo with demeth agents
high dose chemo

Question 10

only cure MDS

Answer 10

allogeneic hematopoietic cell transplantation

Question 11

AML symptoms

Answer 11

neutropenia
anemia
thrombocytopenia

Question 12

diagnosis AML

Answer 12

> 20% bone marrow blasts
flow cytometry– stains: peroxidase, buterate esterase
–determine surface antigen expression: CD13, CD33, CD117

Question 13

genetic analysis AML

Answer 13

classical cytogenetics
FISH
PCR

Question 14

good prognosis AML (genetics and cytogenetics)

Answer 14

genetics- NPM1

cyto- t (8;21), t (15;17), inv (16)

Question 15

poor prognosis AML genetics and cytogenetics

Answer 15

poor- FLT3, KIT, p53

-chrom 5 &7 abnormalities

Question 16

initial treatment AML

Answer 16

stabilize
check heart fx
leukostasis
DIC
tumor lysis
hyperuricemia

Question 17

three phases of treatment of AML

Answer 17

initial
induction- goal is remission
consolidation: 1-4 more courses

Question 18

acute promyelocytic leukemia genetics/cytogenetics

Answer 18

genetics: t(15;17)

PCR-PML/RARa molecular sequence

Question 19

granules in APL contain

Answer 19

plasminogen activators

tumor cell procoagulants- activates VII, and x

Question 20

ATRA

Answer 20

overcomes th edecreased sensitivity to retinoic acid- PML/RAR-a so cells can differentiate

Question 21

ATRA + chemotherapy

Answer 21

induces remission in 80-95%

Question 22

atra side effects

Answer 22

hyperleukocytosis–all cells grow up at once; can result in leukostasis
retinoic acid syndrome–fever, peripheral edema, pulmonary infilitrates