The clinical spectrum of thrombocytosis and thrombocythemia.

Platelet production is the result of a highly ordered maturation of a developmental hierarchy of megakaryocytic progenitor cells regulated by a variety of cytokines. GM-CSF, II-3 and II-6 have a stimulatory effect and several cytokines (TGF-beta, platelet released glycoprotein, platelet factor 4 and interferons) have inhibitory effects down regulating platelet production perhaps as part of an autocrine control loop. Excess platelet production can be clinically characterized as pseudothrombocytosis, thrombocytosis or thrombocythemia; the clinical features and criteria for each are defined. The term thrombocytosis infers its reactive nature and, in the absence of arterial disease or prolonged immobility, it poses little risk regardless of platelet numbers. By contrast, in thrombocythemia, whether primary or associated with other myeloproliferative lesions, significant thrombohemorrhagic events occur. The natural history, rationale, and approach to platelet reduction and control of clinical sequela are reviewed. Clinical therapeutic options include a new agent, Anagrelide.