Essential thrombocythaemia (ET) is a clonal stem cell disorder that frequently presents as an incidental finding of elevated platelet counts. Diagnosis depends on exclusion of other myeloproliferative disorders and reactive thrombocytosis. Patients with platelet counts above 1000 x 10(9)/L should receive platelet-lowering therapy, even if asymptomatic. Definitive recommendations cannot yet be made for asymptomatic patients with lower platelet counts. ET can be treated with alkylating agents, radioactive phosphorus or hydroxyurea, but there is evidence that these agents increase transformation to acute leukaemia. Interferon alfa-2a and anagrelide are useful treatment agents, particularly in younger patients.