Wednesday, 12 May 2010

Marrow Failure - Myelodysplasia


MDS (myelodysplastic sydromes/myelodysplasia)

Acquired clonal d/o fr haemopoetic stem cells

Epid
Pts *> 70 yrs

Ix & Path
Cytopenias - *An
Morphological evid of dysplasia in blood & BM cells
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

Cx
MDS - *AML

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