Developments in the use of plasma exchange and adjunctive therapies to treat immune-mediated thrombotic thrombocytopenic purpura

被引：6

作者：

Picod, Adrien ^{[1
]}

Coppo, Paul ^{[1
,2
,3
]}

机构：

[1] AP HP, Ctr Natl Reference MicroAngiopathies Thrombot, Paris, France

[2] Hop St Antoine, AP HP, Serv Hematol, Paris, France

[3] Sorbonne Univ, Paris, France

来源：

EXPERT REVIEW OF HEMATOLOGY | 2019年 / 12卷 / 06期

关键词：

ADAMTS13; caplacizumab; plasma exchange; plasmapheresis; rituximab; thrombotic microangiopathy; thrombotic thrombocytopenic purpura; von Willebrand factor; VON-WILLEBRAND-FACTOR; FACTOR-CLEAVING PROTEASE; FRESH-FROZEN PLASMA; BLUE-PHOTOINACTIVATED PLASMA; THERAPEUTIC PLASMA; RECOMBINANT ADAMTS13; MICROANGIOPATHIES; RITUXIMAB; EXPERIENCE; MANAGEMENT;

D O I：

10.1080/17474086.2019.1619170

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Introduction: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a life-threatening disease characterized by a severe functional deficit in the von-Willebrand cleaving protease ADAMTS13, due to autoantibody production. The once-dismal prognosis of the disease has been changed by the discovery of the dramatic efficiency of therapeutic plasma exchange (TPE). Areas covered: This review focuses on the history and recent developments in the use of TPE for iTTP with a special emphasis on the consequences for TPE practice of the recent introduction of new highly effective immunosuppressive strategies and anti-von Willebrand factor (vWF) therapies. Expert opinion: Although TPE still represents the cornerstone, emergency treatment of iTTP, their duration, and associated complications could be dramatically reduced in the future by the systematic addition of early immunosuppression using corticosteroids and rituximab as well as an anti-vWF therapy with caplacizumab.

引用

页码：461 / 471

页数：11

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