血栓性血小板减少性紫癜诊断与治疗中国专家共识(2012年版)

2012-11-01 中华血液学杂志,2012,11,33(11):983-984

中文标题:

血栓性血小板减少性紫癜诊断与治疗中国专家共识(2012年版)

发布机构:

发布日期:

2012-11-01

简要介绍:

血栓性血小板减少性紫癜(TTP)为一组微血管血栓出血综合征,其主要临床特征包括微血管病性溶血性贫血、血小板减少、神经精神症状、发热和肾脏受累等。TTP的主要发病机制涉及血管性血友病因子(VWF)裂解蛋白酶(ADAMTSl3)活性缺乏、血管内皮细胞VWF异常释放、血小板异常活化等方面。TTP分为遗传性和获得性两种,后者根据有无原发病分为特发性和继发性。遗传性TTP系ADAMTSl3基因突变导致酶活性降低或缺乏所致,常在感染、应激或妊娠等诱发因素作用下发病。特发性TTP多因患者体内存在抗ADAMTSl3自身抗体(抑制物),导致ADAMTSl3活性降低或缺乏,是主要的临床类型。继发性TTP系因感染、药物、肿瘤、自身免疫性疾病、造血干细胞移植等因素引发,发病机制复杂,预后不佳。

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    2021-12-29 146cdb0fm09暂无昵称

    非常好

    0

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    2019-07-18 枫林5230

    不能下载?

    0