Myeloproliferative disorders

Abstract

The non-leukaemic myeloproliferative disorders are clonal stem cell disorders and have considerable overlap. All these disorders can undergo further clonal evolution to develop either myelofibrosis or acute myeloid leukemia. Venesection is the standard initial therapy of patients with a raised haematocrit. Essential thrombocythaemia remains a diagnosis of exclusion in the absence of a single confirmatory test. The role of hydroxyurea in increasing the risk of acute leukaemia is still far from clear. Further randomised controlled trials are required to define the indications for and the timing of treatment.