PRIMARY THROMBOCYTHEMIA - A CURRENT PERSPECTIVE

The relationships among thrombocytosis, abnormal platelet aggregation and altered hemostasis in primary thrombocythemia remain poorly unders tood. Consequently, the appropriate management of asymptomatic patient s is controversial and needs to be individualized. For symptomatic pat ients, conventional therapy, usually hydroxyurea, is directed primaril y at lowering the platelet count by suppression of megakaryocyte activ ity. Recombinant interferon alpha can selectively lower platelet count s and may offer a reasonable alternative. Recent experience with anagr elide is also most promising in both symptomatic and asymptomatic pati ents. Current thoughts on the pathogenesis and management guidelines a re presented here.