The pathogenesis of essential thrombocythemia as a clonal myeloproliferative disorder has been clearly established. However, there continues to be considerable controversy concerning the management of this disease, particularly because its natural history is consistent with a nearly normal life expectancy. Therapeutic decisions have been complicated by a reliance on largely anecdotal, retrospective experience in the medical literature and, until very recently, an absence of prospective, controlled clinical trials. A recent study of patients with essential thrombocythemia at high risk of thrombosis because of advanced age or previous history of thrombotic complications demonstrated that platelet cytoreduction with hydroxyurea is effective in reducing thrombotic complications. Other cytoreducing agents that have been used in this disease include alkylating agents, recombinant interferon alpha, and anagrelide. The use of antiplatelet therapy is also controversial, and is most highly effective in patients with digital or cerebrovascular ischemic problems.
