Moratelli S, De Sanctis V, Gemmati D, Serino M L, Mari R, Gamberini M R, Scapoli G L
Center for the Study of Haemostasis and Thrombosis, University of Ferrara, Italy.
J Pediatr Endocrinol Metab. 1998;11 Suppl 3:915-21.
Hemostatic parameters of 495 beta-thalassemic patients (421 with thalassemia major and 74 with thalassemia intermedia) were analyzed, to assess their association with the described thrombophilic condition and to verify the role of additional risk factors (e.g. persistent postsplenectomy thrombocytosis, insulin dependent diabetes mellitus, estrogen-progestin treatment and atrial fibrillation). The prevalence of thromboembolic accidents was 5.2% and in four patients (15.3%) inherited or acquired predisposing defects were recognized. The incidence of thromboembolic events and the associated relative risk due to hemocoagulative abnormalities in these patients are discussed.
分析了495例β地中海贫血患者(421例重型地中海贫血和74例中间型地中海贫血)的止血参数,以评估其与所述血栓形成倾向状态的关联,并验证其他危险因素(如脾切除术后持续性血小板增多、胰岛素依赖型糖尿病、雌激素-孕激素治疗和心房颤动)的作用。血栓栓塞事件的发生率为5.2%,在4例患者(15.3%)中发现了遗传性或获得性易患缺陷。讨论了这些患者中血栓栓塞事件的发生率以及由于血液凝固异常导致的相关相对风险。