Hypercoagulability in non-transfusion-dependent thalassemia.
Beta (β)-thalassemia is characterized by a hypercoagulable state and an increased risk of thrombosis, which can result in significant morbidity and mortality. The molecular and cellular mechanisms contributing to hypercoagulability are diverse and include chronic platelet activation, alteration of red blood cell membranes, abnormal expression of adhesion molecules on vascular endothelial cells, and dysregulation of hemostasis. Regular transfusions decrease the risk of thrombosis, whereas splenectomy significantly increases the risk. Splenectomized adults with non-transfusion-dependent thalassemia are also at high risk for ischemic brain damage. Strategies to lower the risk of thrombosis should be considered, including transfusion therapy to raise hemoglobin levels and avoidance or delay of splenectomy.
IRCCS Ca' Granda Foundation Maggiore Policlinico Hospital, University of Milan, Via Francesco Sforza 35, Milan, Italy. maria.cappellini@unimi.it
Maria D
MD
Cappellini
Khaled M
KM
Musallam
Erika
E
Poggiali
Ali T
AT
Taher
D001803:Blood Transfusion; D006801:Humans; D012307:Risk Factors; D019851:Thrombophilia / Q000097:blood*; D017086:beta-Thalassemia / Q000097:blood*