Myeloproliferatic Syndromes and AML Flashcards
myelodysplastic syndromes
clonal disorder from HSC lesion –>
dysfunctional cells and premature apoptosis
peripheral cytopenias despite hyper cellular bone marrow
bone marrow biopsy in MDS
normo or hypocellular
increased myeloblasts
abnormal localization
increased iron (ringed sideroblasts, increased iron storage)
types of MDS and #s of blasts in BM
20% AML
IPSS scoring system
for MDS - higher score = bad
3 factors: 1. # blasts in marrow, 2. # cytopenias, 3. karyotype
tx of MDS
supportive care growth factors immunomodulation chemo w/ demethylating agents high dose chemo (induction allogeneic HSC transplant
sx of AML
cytopenias
- neutropenia – infection
- anemia – fatigue, dyspnea
- thrombocytopenia - bleeding, bruising
AML dx
> 20% bone marrow blasts!
cytochrome stains, flow cytometry
BMB in AML
hypercellular
>20% blasts - prominent nucleoli, high nucleus-to-cytoplasm ratio, AUER RODS
cytochemical analysis: MPO+
flow cytometry markers - B cell lineage AML
CD19, 20, 22
flow cytometry markers - T cell lineage AML
CD3, 4
flow cytometry markers - myeloid lineage AML
CD33
favorable karyotypes in AML
Inv(16)
t(15;17)
t(8:21)
bad karyotypes in AML
trisomy 8, monosomy 7, del 5q
what drugs cause AML/MDS?
alkalating agents
topoisomerase type II
MPO+
indicates AML
tx for AML
induction - myeloablative, goal is remission
consolidation - 1-4 more courses of chemo after remission
allogeneic stem cell transplant
acute promyelocytic leukemia
cytogenetics: t(15,17), PML/RAR-alpha molecualr sequence
coagulopathies, bleeding common –> DIC
tx for APL
ATRA - encourages maturation and apoptosis of malignant blasts
Arsenic - degrades PML/RAR-alpha protein –> differentiation and apoptosis of APL blasts
ATRA side effects
hyperleukocytosis –> all WBCs grow up at once
retinoic acid syndrome: fever, peripheral edema, resp distress - occurs in 25% pts, can be fatal
