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How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

Scully, M; Goodship, T; (2014) How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome. British Journal of Haematology , 164 (6) 759 - 766. 10.1111/bjh.12718. Green open access

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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.

Type: Article
Title: How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/bjh.12718
Publisher version: http://dx.doi.org/10.1111/bjh.12718
Language: English
Additional information: © 2014 The Authors. British Journal of Haematology Published by John Wiley & Sons Ltd. This is an open access article under the terms of the Creative Commons Attribution-NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes. http://creativecommons.org/licenses/by-nc/3.0/
Keywords: thrombotic thrombocytopenic purpura, atypical haemolytic uraemic syndrome, treatment, diagnosis, complement
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > Institute of Cardiovascular Science
URI: https://discovery.ucl.ac.uk/id/eprint/1469779
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