ERYTHROMELALGIC, THROMBOTIC AND HEMORRHAGIC MANIFESTATIONS IN 50 CASES OF THROMBOCYTHEMIA

Fifty consecutive patients with thrombocythemia (35 men and 15 women) were diagnosed as primary thrombocythemia (PT) in 30 and thrombocythem ia associated with polycythemia vera (PV) in 20. The symptoms were pla telet-mediated erythromelalgia in 16 PT and 15 PV, coronary artery dis ease in 3 PT and 2 PV, atypical cerebral ischemic attacks in 8 PT and 3 PV, paradoxical thrombosis and bleeding in 3 PT and 2 PV and hemorrh ages alone in 6 PT and 2 PV patients. Erythromelalgia was localized in the forefoot sole and toes in 28, the fingertips in 9, the handpalm i n 2. Untreated erythromelalgia progressed to acrocyanosis or periphera l ischemia with necrosis in a toe or fingertip in 14 cases. Painful re d, warm and indurated erythromelalgic hot spots in the skin of the upp er legs were misdiagnosed as superficial thrombophlebitis in 5 PT and 2 PV patients. Erythromelalgia in thrombocythemia already occurred at slightly increased platelet counts above 400 x 10(9)/l. The curative e ffect of aspirin on erythromelalgia in thrombocythemia was consistentl y accompanied by a significant increase of platelet counts. Erythromel algia and bleeding paradoxically occurred in 5 patients at platelet co unts between 1000 and 2000 x 10(9)/l. In this situation aspirin preven ts erythromelalgic and microcirculatory circulation disturbances, but further increases the risk of serious bleeding complications. Presenti ng hemorrhagic manifestations in thrombocythemia were observed at plat elet counts in excess of 1000 x 10(9)/l in 9 PT and 4 PV patients as s evere epistaxis in 5, atypical ecchymoses in 3, gastrointestinal bleed ing in 2 and secondary bleeding in 3. The concept of platelet-mediated erythromelalgia, thrombosis and hemorrhages in thrombocythemia is dis cussed.