Abstract
As the life expectancy of β-thalassemia patients has markedly improved over the last few decades, several manifestations are increasingly recognized. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia patients, has led to the identification of a hypercoagulable state in thalassemia. In this review, the current clinical experience attributed to the coagulopathy in thalassemia intermedia patients is summarized. Recommendations for thrombosis prophylaxis are also discussed.
Highlights
As the life expectancy of β-thalassemia patients has markedly improved over the last few decades, several manifestations are increasingly recognized
Recent evidence suggests that the diagnosis of β-thalassemia rc intermedia (TI) carries higher morbidity than previously recognized, especially in the transfusion-independent patient where the mechae nism of disease remains largely unbalanced.[1]
After examining data from 8,860 patients in the Mediterranean area and Iran, Taher et al observed that thromboembolic events (TEE) occurred 4.38 times (95% confidence interval [CI] 3.14 – 6.10, P < 0.001) more frequently in thalassemia intermedia (TI) than thalassemia major (TM), with more venous events occurring in TI and more arterial events occurring in TM.[8]
Summary
Recent evidence suggests that the diagnosis of β-thalassemia rc intermedia (TI) carries higher morbidity than previously recognized, especially in the transfusion-independent patient where the mechae nism of disease remains largely unbalanced.[1] Three main factors highm light the pathophysiology of TI, ineffective erythropoiesis, chronic anemia/hemolysis, and iron overload secondary to increased intestinal m absorption.[1] Among the medical complications of TI that were found to o occur at high rates, even more frequent than patients with TM, are n-c Correspondence: A.T. Taher, Professor of Medicine Hematology-Oncology o Associate Chair, Research Department of Internal Medicine, American N University of Beirut Medical Center, Beirut, Lebanon. Thalassemia Intermedia Management Aiming for Lowering Complication rates Across a Region of Endemicity (OPTIMAL CARE) study evaluated 584 patients with TI at six comprehensive care centers (Lebanon, Italy, Iran, Egypt, United Arab Emirates, and Oman) for [page 56]
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