Myelodysplastic Syndromes

The answer first

The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal haematopoietic stem cell neoplasms characterised by ineffective haematopoiesis, peripheral cytopenia, dysplastic morphology, and a variable risk of transformation to acute myeloid leukaemia. They are predominantly diseases of older adults (median age about 75 years) and their management spans supportive care (transfusion, iron chelation, growth factors), disease-modifying therapy (hypomethylating agents, lenalidomide, luspatercept), and curative allogeneic haematopoietic stem cell transplantation in carefully selected patients. ^{[2] [5]}

The FRACP candidate must be able to defend three positions without hedging: (1) the diagnosis of MDS requires both morphological dysplasia and the exclusion of secondary causes of cytopenia and dysplasia (B12 deficiency, alcohol, viral infection, drug effect, copper deficiency); (2) prognostic stratification (IPSS-R, IPSS-M) integrates cytopenia, blast percentage, and cytogenetics, and drives the treatment decision between supportive care, hypomethylating therapy, and transplantation; and (3) allogeneic stem cell transplantation is the only curative therapy but is offered only to patients with a high-risk disease, an adequate performance status, and a suitable donor. Lead with the decision, then the evidence, then the trap. ^{[5] [6]}

References9ShowHide

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