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血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)的发病机制直到ADAMTS13的发现才揭开了超过半世纪的谜底.ADAMTS13主要合成于肝脏,它主要的功能是特异性切割锚定在内皮细胞表面、血液循环中以及血管损伤部位的血管性血友病因子(von Willebrand factor,VWF).当由于ADAMTS13基因突变或存在ADAMTS13自身抗体而导致血浆中ADAMTS13活性缺失(20%).现今初治方案仍然选择血浆输注或血浆置换,但新的治疗方法如重组的ADAMTS13和基因治疗仍在研制中.另外ADAMTS13活性的缺失已经被认为是发展为心肌梗死、中风、脑型疟疾和先兆子痫的一个风险因素.“,”The pathogenesis of thrombotic thrombocytopenic purpura (TTP) had been unveiled until ADAMTS13 was found more than half a century. ADAMTS13 is mainly synthesized in the liver, and its main function is to cleave von Willebrand factor (VWF) anchored on the surface of endothelial cells, in blood circulation, and vascular injury sites. Defective ADAMTS13 activity in plasma (20%). The initial treatment still is plasma infusion or plasma exchange, but the new methods of recombinant ADAMTS13 and gene therapy are still under development. In addition, ADAMTS13 deficiency has been considered as a risk factor for myocardial infarction, stroke, cerebral malaria andpreeclampsia.