Abstract

MACEDO, Joana; COSTA, Emília; BARBOT, José  and  NETO, Cláudia. Polycythemia vera: a case report. Nascer e Crescer [online]. 2015, vol.24, n.2, pp.83-87. ISSN 0872-0754.

Polycythemia vera (PV) is a myeloproliferative disturbance of haematopoietic cells characterized by abnormal and overstated production of erythrocytes, leukocytes and platelets. Other disease features include splenomegaly, thrombohemorrhagic complications, vasomotor disturbances, pruritus and a small risk of disease progression into acute myeloid leukemia or myelofibrosis. Thrombosis is the presenting symptom in 20% of patients with PV. It is a rare disease with an incidence of 2.3/100.000 people per year, and is even more uncommon in children and adolescents. We present a case report of a fourteen-year-old years old adolescent with clinical and laboratorial findings suggestive of polycythemia vera. Treatment with alpha-interferon was initiated. Erythrocyte and platelet count are now in the normal range. The authors make, in the context of this case report, a brief review of the criteria for the differential diagnosis of reactive thrombocytosis and myeloproliferative diseases, manifestations and treatment options.

Keywords : Adolescence; Polycythemia vera; Thrombosis; Thrombocytosis.

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