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Acquired thrombotic thrombocytopenic purpura with isolated CFHR3/1 deletion—rapid remission following complement blockade

BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the abundance of uncleaved ultralarge von Willebrand factor multimers (ULvWF) due to acquired (autoantibody-mediated) or congenital vWF protease ADAMTS13 deficiency. Current treatment recommendations include plasma exchange therapy a...

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Detalles Bibliográficos
Autores principales: Bitzan, Martin, Hammad, Rawan M., Bonnefoy, Arnaud, Al Dhaheri, Watfa Shahwan, Vézina, Catherine, Rivard, Georges-Étienne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6019431/
https://www.ncbi.nlm.nih.gov/pubmed/29728803
http://dx.doi.org/10.1007/s00467-018-3957-8