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How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

Lookup NU author(s): Professor Tim Goodship

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Abstract

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.

Publication metadata

Author(s): Scully M, Goodship T

Publication type: Review

Publication status: Published

Journal: British Journal of Haematology

Year: 2014

Volume: 164

Issue: 6

Pages: 759-766

Print publication date: 01/03/2014

Online publication date: 06/01/2014

ISSN (print): 0007-1048

ISSN (electronic): 1365-2141

Publisher: WILEY-BLACKWELL

URL: http://dx.doi.org/10.1111/bjh.12718

DOI: 10.1111/bjh.12718

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