THROMBOEMBOLIC EVENTS IN BETA-THALASSEMIA MAJOR - AN ITALIAN MULTICENTER STUDY

Thromboembolic (TE) events have been frequently reported in beta-thala ssemic patients in association with known risk factors such as diabete s, complex cardiopulmonary abnormalities, hypothyroidism, liver functi on anomalies, and postsplenectomy thrombocytosis. In a recent survey i nvolving 9 Italian thalassemic centers, we identified 32 patients with TE episodes in a total of 735 subjects, of whom 683 had thalassemia m ajor and 52 thalassemia intermedia, corresponding to 3.95 and 9.61%, r espectively. There was a great variation in localization: the main one (16/32) was CNS, with a clinical picture of headache, seizures and he miparesis. Other localizations were the pulmonary (3 patients), mesent eric (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). In tracardiac thrombosis was found in 2 subjects and clinical and laborat ory 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 f unction anomalies, hypothyroidism) than those without TE events (50 vs . 13.8%), we suggest close monitoring of those patients who are at hig her risk of developing TE events because of the presence of one or mor e of these predisposing factors.