The hypercoagulable state in thalassemia

Abstract

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. (Blood. 2002;99:36-43)

2-Butoxyethanol Female-Rat Model of Hemolysis and Disseminated Thrombosis: X-Ray Characterization of Osteonecrosis and Growth-Plate Suppression

“…The target organs for thrombosis and infarction found in this model, however, are similar to those observed in human disorders characterized by hemolysis and thrombosis, such as thalassemia and sickle cell disease (Smith, 1996;Eldor and Rachmilewitz, 2002;Ezov et al, 2002;Shabat et al, 2004). Furthermore, enhanced red blood cell and endothelial cell interaction has been demonstrated both in this animal model and human thrombotic diseases Yedgar et al, 1999;Koshkaryev et al, 2003).…”

Pulmonary hypertension in patients with hematological disorders following splenectomy

“…Splenectomised patients have higher plasma hemoglobin level than non-splenectomised thalassemia intermedia and higher circulating hemoglobin containing vesicles suggest that worsening of pulmonary hypertension after splenectomy may be due to increased cell free plasma haemoglobin [12]. Moreover, hyper coagulable state in thalassemia is already well established [12,14]. Pulmonary thromboembolism was also found in a large number of our splenectomised as well non-splenectomised patients with hemolytic anemia this has been demonstrated by other workers too [7,15].…”

Does Profile of Hemoglobin Eβ-thalassemia Patients Change After Splenectomy? Experience of a Tertiary Thalassemia Care Centre in Eastern India

“…Tripatara et al [20] demonstrated the existence of a low grade consumptive coagulopathy among transfused splenectomized patients with severe clinical manifestations, indicating that these patients may have a higher risk for thrombosis than comparable patients with intact spleen. Prothrombotic hemostatic anomalies, including low levels of coagulation inhibitors, such as protein C and protein S as well as thrombocytosis and platelet activation, have also been observed in these patients [21]. Cardiac ejection fractions and pulmonary artery pressures were measured by 2D transthoracic echocardiography and Doppler study.…”