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Blood, 1 January 2002, Vol. 99, No. 1, pp. 36-43
REVIEW ARTICLE
The hypercoagulable state in thalassemia
Amiram Eldor and
Eliezer A. Rachmilewitz
From the Institute of Hematology, Tel-Aviv Sourasky
Medical Center, Sackler Faculty of Medicine, Tel-Aviv University,
Tel-Aviv, Israel; and Department of Hematology, Edith Wolfson Medical
Center, Holon, Israel.
Thalassemia is a congenital hemolytic disorder caused by a
partial or complete deficiency of  - or  -globin chain synthesis. Homozygous carriers of -globin gene defects suffer from severe anemia and other serious complications from early childhood. The disease is treated by chronic blood transfusion. However, this can
cause severe iron overload resulting in progressive organ failure. Some
forms of thalassemia are also associated with a similar clinical
picture. Despite the difficulties associated with treatment, standards
of care for thalassemic patients have improved in recent years,
resulting in almost doubling of the average life expectancy. As a
consequence, additional previously undescribed, complications are now
being recognized. In particular, profound hemostatic changes have been
observed in patients with -thalassemia major (-TM) and
-thalassemia intermedia (-TI) and also in patients with thalassemia (hemoglobin H disease). The presence of a higher than
normal incidence of thromboembolic events, mainly in -TI, and the
existence of prothrombotic hemostatic anomalies in the majority of the
patients, even from a very young age, have led to the recognition of
the existence of a chronic hypercoagulable state in thalassemic
patients. Despite the appearance of numerous publications on the
frequent occurrence of thromboembolic complications in thalassemia,
this complication has not been emphasized or comprehensively reviewed.
This review summarizes the current literature and discusses possible
mechanisms of the lifelong hypercoagulable state that exists in thalassemia.
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