Flashcards in Myelodysplastic Syndromes & AML Deck (23)
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Myelodysplastic Syndrome
mutated cell that divide but do not mature normally
results in
-dysfunctional cells and premature apoptosis
-peripheral cytopenias despite hypercellular bone marrow
1
peripheral lab findings in MDS
cytopenias
myeloid: left-shift
erythrocytes- macrocytosis
platelets- large, hypogranular
monocytosis
2
bone marrow in MDS
normo/hypercellular (ineffective hematopoiesis)
increase myeloblasts
dyspoiesis in 1 or more cell lines
abnormal localization
increased iron (sideroblasts)
3
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
6
suspected causes for secondary/treatment-related MDS
younger age of onset
cancer treatment modalities
chromosomal abnormalities
poor prognosis
7
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
10
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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