How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

被引：122

作者：

Scully, Marie ^{[1
]}

Goodship, Tim ^{[2
]}

机构：

[1] UCLH, Dept Haematol, London W1T 4EU, England

[2] Newcastle Univ, Dept Renal Med, Inst Med Genet, Newcastle Upon Tyne NE1 7RU, Tyne & Wear, England

来源：

BRITISH JOURNAL OF HAEMATOLOGY | 2014年 / 164卷 / 06期

基金：

英国医学研究理事会;

关键词：

thrombotic thrombocytopenic purpura; atypical haemolytic uraemic syndrome; diagnosis; complement; treatment; COMPLEMENT INHIBITOR ECULIZUMAB; CLEAVING PROTEASE ADAMTS-13; UK TTP REGISTRY; PLASMA-EXCHANGE; RITUXIMAB; EFFICACY; SAFETY; DGKE; MICROANGIOPATHIES; TRANSPLANTATION;

D O I：

10.1111/bjh.12718

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment. They are defined by microangiopathic haemolytic anaemia and thrombocytopenia, with renal involvement primarily in aHUS and neurological and cardiological sequelae in TTP. Prompt treatment for most cases of both conditions is with plasma exchange initially and monoclonal therapy (rituximab in TTP and eculizumab in aHUS) as the mainstay of therapy. Here we discuss the diagnosis and therapy for both disorders.

引用

页码：759 / 766

页数：8

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