2014, Number 4
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Rev Cubana Hematol Inmunol Hemoter 2014; 30 (4)
Polycythemia vera and thrombophilia
Macías PI, Fernández DND, Castillo GD, Fundora ST
Language: Spanish
References: 12
Page: 381-386
PDF size: 87.35 Kb.
ABSTRACT
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.
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