Recent progress in the pathogenesis and management of essential thrombocythemia

In the last decade, the diagnosis of essential thrombocythemia (ET) has been refined by appreciation of the occurrence of karyotypically occult but molecularly evident chronic myelogenous leukemia and morphologically subtle myelodysplastic syndrome (MDS) and cellular-phase agnogenic myeloid metaplasia (AMM). Although ET continues to be defined by the presence of nonreactive thrombocythemia that is not accounted for by another chronic myeloid disorder, recent studies of clonality and other laboratory parameters have suggested clinically relevant biologic heterogeneity among affected patients. Furthermore, randomized, prospective, and controlled retrospective data have provided additional clinical information that has resulted in the development of risk categories and risk-adjusted treatment recommendations.