Acquired clonal d/o fr haemopoetic stem cells
Epid
Pts *> 70 yrs
Ix & Path
Cytopenias - *An
Epid
Pts *> 70 yrs
Ix & Path
Cytopenias - *An
Morphological evid of dysplasia in blood & BM cells
BM - cellular/Hcellular (NB difference to AA)
BM - cellular/Hcellular (NB difference to AA)
Aet
*idiopath
Prev cytotoxic CTx/RTx
Dx
Typical dysplastic changes of Blood & BM
+ Clonal cytogenetic abnormalities on chromosomal examination of BM cells
Prognosis
3 variables
- % immat blast cells in BM (low blast = best)
- cytogen abnorms
- no of cytopenias
Tx
- Supportive - red cell transfusion, iron chelation Tx after multiple transfusions, recombinant erythropoetin therapy
- Cytotoxic CTx - for AML, h/e poor response rate
- Differentiation therapy eg 5-azacytidine (accelerates differentiation of MDS clone, so normal stem cells can regain dominance)
- StC transplantion - definitive cure; only poss 4 small fraction of younger pts
- Cytotoxic CTx - for AML, h/e poor response rate
- Differentiation therapy eg 5-azacytidine (accelerates differentiation of MDS clone, so normal stem cells can regain dominance)
- StC transplantion - definitive cure; only poss 4 small fraction of younger pts
Cx
MDS - *AML
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