Thromboembolic Events in Beta Thalassemia Major: An Italian Multicenter StudyBorgna Pignatti C.a · Carnelli V.b · Caruso V.c · Dore F.d · De Mattia D.e · Di Palma A.f · Di Gregorio F.c · Romeo M.A.c · Longhi R.b · Mangiagli A.g · Melevendi C.h · Pizzarelli G.c · Musumeci S.c
Departments of Pediatrics, University of a Verona, b Milano, c Catania, d Sassari, e Bari, f Ferrara, g Siracusa, h Genova, Italy
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Thromboembolic (TE) events have been frequently reported in β-thalassemic patients in association with known risk factors such as diabetes, complex cardiopulmonary abnormalities, hypothyroidism, liver function anomalies, and postsplenectomy thrombocytosis. In a recent survey involving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia major and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, respectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and hemiparesis. Other localizations were the pulmonary (3 patients), mesenteric (1 patient) and portal (2 patients) sites. There were 6 cases of deep venous thrombosis (2 in the upper limbs, 4 in the lower ones). Intracardiac thrombosis was found in 2 subjects and clinical and laboratory signs of DIC were observed in 2 others during pregnancy. Since our patients with TE events present a statistically significantly higher incidence of associated dysfunction (cardiomyopathy, diabetes, liver function anomalies, hypothyroidism) than those without TE events (50 vs. 13.8%), we suggest close monitoring of those patients who are at higher risk of developing TE events because of the presence of one or more of these predisposing factors.
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