Resumen

MACIAS PEREZ, Ivis; FERNANDEZ DELGADO, Norma D; CASTILLO GONZALEZ D, Dunia  y  FUNDORA SARRAFF, Teresa. Polycythemia vera and thrombophilia. Rev Cubana Hematol Inmunol Hemoter [online]. 2014, vol.30, n.4, pp. 381-386. ISSN 0864-0289.

Polycythemia vera is a chronic myeloproliferative syndrome resulting from abnormal proliferation of the pluripotent stem cell, giving rise to a clonal hematopoiesis, with predominant erythroid hyperplasia over other hematopoietic lines. In this as in other myeloproliferative syndromes thrombotic and hemorrhagic complications are observed, which are cause of morbidity and mortality in this group of patients.  The association of polycythemia vera and thrombophilic states is infrequent and when it appears tendency of thrombus formation is increased. Furthermore, thrombophilias are a heterogeneous group of conditions associated to an increased risk of arterial and venous thrombosis where a group of risk factors exists.  We present a 50 year-old male patient with a diagnosis of polycythemia vera 12 years ago, who has been treated with hydroxyurea and recombinant alpha interferon.  During the last two years he started suffering recurrent bleeding episodes in both upper and lower limbs also presenting a deep vein thrombosis. Hemostasis studies were performed and a significant reduction in the activity of proteins C and S were detected, which association is infrequently described, concomitant with alterations of platelet function. This case demonstrates the importance of searching for the presence of thrombophilic states associated to hematological malignancies.

Palabras clave : polycythemia vera; thrombosis; thrombophilias; alterations in platelet function.

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