[Essential thrombocythemia: a 35-year experience and review of the literature]

Essential thrombocythemia (ET) is a clonal myeloproliferative disorder characterized by an uncontrolled rise in peripheral blood platelet count. The aim of this report was to determine the clinical and laboratory data of this disease in a 35 years revision. Of the patients with the diagnosis of ET, we selected those who fulfilled five of the six diagnostic criteria proposed by the Poli Vera Study Group. We found 14 cases (10 female and 4 male) with a median age of 54.5 years (range 29-74). The most frequent initial clinical finding was hemorrhage and in four cases the diagnosis was preoperative. Median platelet count was 1,355 x 10(9)/L (range 600 to 3,750). One case had iron deficiency which was corrected before ET was diagnosed. None has evolved to acute leukemia. Initially, most of the cases were treated with busulphan and two received alpha-interferon which was promptly changed to busulphan because of secondary effects. Three patients have died due to hemorrhagic complications and one due to thrombosis. ET has a low frequency in our country and must be considered an exclusion diagnosis. Iron deficiency may mask the diagnosis specially in the cases with a platelet count not very high. Treatment can provide in general a long survival of good quality of life.