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How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome
Author(s) -
Scully Marie,
Goodship Tim
Publication year - 2014
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.12718
Subject(s) - haemolytic uraemic syndrome , thrombotic thrombocytopenic purpura , medicine , atypical hemolytic uremic syndrome , schistocyte , immunology , purpura (gastropod) , platelet , dermatology , biology , antibody , complement system , ecology , biochemistry , escherichia coli , gene
Summary 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 a HUS 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 a HUS ) as the mainstay of therapy. Here we discuss the diagnosis and therapy for both disorders.