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]

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Summary

Coagulation and thrombotic risk in thalassemia intermedia

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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