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Br J Haematol. 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.

Author information

1
Department of Haematology, University College London Hospital, London, UK.

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

PMID:
24387053
PMCID:
PMC4163720
DOI:
10.1111/bjh.12718
[Indexed for MEDLINE]
Free PMC Article

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