[血栓性血小板减少性紫癜的最新进展]
[Update on thrombotic thrombocytopenic purpura].
作者信息
Schleinitz N, Poullin P, Camoin L, Veit V, Bernit E, Mazodier K, Lefèvre P, Dignat-George F, Kaplanski G, Durand J M, Harlé J R
机构信息
Service de médecine interne, CHU La-Conception, AP-HM, Marseille cedex 5, France.
出版信息
Rev Med Interne. 2008 Oct;29(10):794-800. doi: 10.1016/j.revmed.2007.09.034. Epub 2007 Oct 22.
PURPOSE
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder and early treatment is vital. Here, we review the recent advances in the understanding of the pathophysiology of TTP and its treatment.
CURRENT KNOWLEDGE AND KEY POINTS
Recent advances have shown that TTP is caused by deficiency of the (ADAMTS-13) metalloprotease that cleaves von Willebrand factor multimers. Acquired TTP is associated to inhibitory antibodies directed against ADAMTS-13. This has led to assess new therapeutic approaches in refractory and relapsing forms of TTP and the use of rituximab has shown very encouraging results.
FUTURE PROSPECTS AND PROJECTS
A better characterization of TTP amongst the other thrombotic microangiopathies has allowed the use of new therapeutic approaches with the use of rituximab. The encouraging results reported with rituximab in some forms of TTP challenge the classic treatment based on plasma exchanges.
目的
血栓性血小板减少性紫癜（TTP）是一种危及生命的疾病，早期治疗至关重要。在此，我们综述了对TTP病理生理学及其治疗认识的最新进展。
当前知识与要点
最新进展表明，TTP是由裂解血管性血友病因子多聚体的金属蛋白酶（ADAMTS - 13）缺乏所致。获得性TTP与针对ADAMTS - 13的抑制性抗体有关。这促使人们评估针对难治性和复发性TTP的新治疗方法，而利妥昔单抗的使用已显示出非常令人鼓舞的结果。
未来前景与计划
对TTP与其他血栓性微血管病更好的特征描述使得利用利妥昔单抗采用新的治疗方法成为可能。利妥昔单抗在某些形式的TTP中报告的令人鼓舞的结果对基于血浆置换的传统治疗提出了挑战。
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