SICKLE CELL DISEASE AND VENOUS THROMBOEMBOLISM
Author(s) -
Zohreh Rahimi,
Abbas Parsian
Publication year - 2011
Publication title -
mediterranean journal of hematology and infectious diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.682
H-Index - 31
ISSN - 2035-3006
DOI - 10.4084/mjhid.2011.024
Subject(s) - medicine , pathogenesis , hemostasis , disease , venous thromboembolism , thalassemia , mutation , sickle cell anemia , gastroenterology , thrombosis , pediatrics , genetics , gene , biology
Hemoglobin S in homozygous state or in combination with one of the structural variants of Hb D-Punjab, Hb O-Arab, Hb C or β-thalassemia mutation results in sickle cell disease (SCD) that is characterized by chronic hemolytic anemia and tissue injury secondary to vasooclusion. A chronic hypercoagulable state in SCD has been established with the increased risk of thromboembolic complications in these patients. The goal of present review is to survey of the literature related to thromboembolic events and genetic risk factors involved in the manifestation of these events in SCD patients with focus on studies from Mediterranean countries. Also, this review covers the pathogenesis of hypercoagulability and alteration in the components of hemostasis system.
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