Visudhiphan S, Ketsa-Ard K, Tumliang S, Piankijagum A
Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Southeast Asian J Trop Med Public Health. 1994 Sep;25(3):449-56.
In beta-Thalassemia hemoglobin E (beta-thal Hb E), hypoxemia with abnormal lung function was described and postmortem examination in these patients showed organized pulmonary trombi with thickened arterial wall, particularly in post-splenectomized cases. Coagulation and platelet profiles were studied in 58 beta-thal Hb E patients. In 35 cases with intact spleen, the fibrinolytic activity was significantly decreased with high antithrombin III activity, while coagulation tests revealed mild abnormality. The platelet aggregation to ADP, adrenaline, collagen and ristocretin were defective and platelet 5-hydroxytryptamine content was lower than normal. Twenty-three patients who had been splenectomized for 5-18 years, decreased fibrinolytic activity and high antithrombin III activity were also observed. The coagulation profiles and platelet aggregation in response to ADP, adrenaline and collagen showed better results. Fourteen cases exhibited thrombocytosis and their thrombin generation was in the hypercoagulable range. Platelet aggregation in response to ristocetin remained defective and platelet 5-hydroxytryptamine content was lower than in cases with intact spleens. Defective aggregation to ristocetin would indicate abnormal von Willebrand's factor (vWF). Decreased fibrinolysis should very likely have a role in the occurrence of thrombosis and the better hemostatic profiles in post-splenectomized cases would contribute to the more frequent thrombotic incidence in these cases.
在β地中海贫血血红蛋白E（β-地中海贫血Hb E）中，曾有低氧血症伴肺功能异常的描述，对这些患者进行的尸检显示存在机化性肺血栓，动脉壁增厚，尤其是在脾切除术后的病例中。对58例β-地中海贫血Hb E患者的凝血和血小板情况进行了研究。在35例脾脏完整的病例中，纤维蛋白溶解活性显著降低，抗凝血酶III活性升高，而凝血试验显示轻度异常。血小板对二磷酸腺苷（ADP）、肾上腺素、胶原和瑞斯托霉素的聚集功能存在缺陷，血小板5-羟色胺含量低于正常水平。23例已行脾切除术5至18年的患者，也观察到纤维蛋白溶解活性降低和抗凝血酶III活性升高。凝血情况以及血小板对ADP、肾上腺素和胶原的聚集反应显示出较好的结果。14例出现血小板增多症，其凝血酶生成处于高凝范围。血小板对瑞斯托霉素的聚集功能仍然存在缺陷，且血小板5-羟色胺含量低于脾脏完整的病例。对瑞斯托霉素聚集功能缺陷表明血管性血友病因子（vWF）异常。纤维蛋白溶解降低很可能在血栓形成中起作用，而脾切除术后病例中较好的止血情况会导致这些病例中血栓形成发生率更高。
