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How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

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 pri...

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Detalles Bibliográficos
Autores principales: Scully, Marie, Goodship, Tim
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wiley-Blackwell 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4163720/
https://www.ncbi.nlm.nih.gov/pubmed/24387053
http://dx.doi.org/10.1111/bjh.12718
Descripción
Sumario: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.