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血栓性血小板减少性紫癜(thrombotic thrombocytopemic purpura,TTP)是一组少见的、十分严重的危及生命的血栓性微血管病,以微血管溶血性贫血、外周血小板减少和严重的血管性血友病因子(VWF)蛋白裂解酶ADAMTS13缺乏为特征。治疗重点是通过血浆置换、应用利妥昔单抗和糖皮质激素来增加ADAMTS13活性、清除或抑制ADAMTS13抗体。在临床中,准确地做出诊断并尽早启动治疗能够显著提高患者的生存率。
Thrombotic thrombocytopemic purpura (Thrombotic thrombocytopemic purpura, TTP) is a rare, very serious life-threatening thrombotic microvascular disease with microvascular hemolytic anemia, peripheral thrombocytopenia and severe von Willebrand factor (VWF ) Lysozyme ADAMTS13 deficiency is characterized. The focus of treatment is to increase ADAMTS13 activity by plasma exchange, rituximab and glucocorticoids to eliminate or inhibit ADAMTS13 antibodies. In clinical practice, accurate diagnosis and early initiation of treatment can significantly improve patient survival.