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Flashcards in Myelodysplastic Syndromes & AML Deck (23):
0

Myelodysplastic Syndrome

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

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peripheral lab findings in MDS

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

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bone marrow in MDS

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

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abnormal localization in bone marrow

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

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Myelodysplastic Syndromes are NOT

curable

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suspected causes for primary MDS

cigarrete smoke
toxic chemicals
radiation

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suspected causes for secondary/treatment-related MDS

younger age of onset
cancer treatment modalities
chromosomal abnormalities
poor prognosis

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International Prognostic Scoring System ranges from

0-3.5

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prognosis depends on 3 factors

number of blasts in marrow
number of cytopenias
karotype

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treatment MDS

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

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only cure MDS

allogeneic hematopoietic cell transplantation

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AML symptoms

neutropenia
anemia
thrombocytopenia

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diagnosis AML

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

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genetic analysis AML

classical cytogenetics
FISH
PCR

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good prognosis AML (genetics and cytogenetics)

genetics- NPM1
cyto- t (8;21), t (15;17), inv (16)

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poor prognosis AML genetics and cytogenetics

poor- FLT3, KIT, p53
-chrom 5 &7 abnormalities

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initial treatment AML

stabilize
check heart fx
leukostasis
DIC
tumor lysis
hyperuricemia

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three phases of treatment of AML

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

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acute promyelocytic leukemia genetics/cytogenetics

genetics: t(15;17)
PCR-PML/RARa molecular sequence

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granules in APL contain

plasminogen activators
tumor cell procoagulants- activates VII, and x

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ATRA

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

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ATRA + chemotherapy

induces remission in 80-95%

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atra side effects

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