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Review
. 2014 Mar;164(6):759-66.
doi: 10.1111/bjh.12718. Epub 2014 Jan 6.

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

Marie Scully  1 , Tim Goodship
Affiliations
Free PMC article
Review

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

Marie Scully et al. Br J Haematol. 2014 Mar.
Free PMC article

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.

Keywords: atypical haemolytic uraemic syndrome; complement; diagnosis; thrombotic thrombocytopenic purpura; treatment.

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Figures

Figure 1
Figure 1
Summary in the diagnosis and treatment of TTP and aHUS. TTP, thrombotic thrombocytopenic purpura; (a)HUS (atypical) haemaolytic uraemic syndrome; STES‐HUS, Shiga‐like toxin‐producing E. coli haemaolytic uraemic syndrome; TMA, thrombotic microangiopathy; PEX, plasma exchange; ADAMTS13, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; IgG, immunoglobulin G.
See this image and copyright information in PMC

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