The ICD classification of the myelodysplastic syndromes (MDS), and the four main features of the descriptive epidemiology of the condition are discussed. MDS is: (1) a rare disease; which may be, (2) on the increase; (3) which primarily affects the elderly; and (4) which predominantly affects men. We reviewed four causal models pertaining to the relationship between MDS and leukemia. These models may be described as: (1) non-biological correlates; (2) biological correlates; (3) early phase; and (4) interactive cause. Each model is described and the evidence in support of or against each is presented.
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