Blood Transfusion - 3 2010

Rituximab for managing relapsing or refractory patients with idiopathic thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome
 
Authors:  Domenica Caramazza, Gerlando Quintini, Ignazio Abbene, Lucio Lo Coco, Alessandra Malato, Rosa Di Trapani, Giorgia Saccullo, Giuseppina Pizzo, Roberto Palazzolo, Rita Barone, Giuseppina Mazzola, Sergio Rizzo, Paolo Ragonese, Paolo Aridon, Vincenzo Abbadessa, Sergio Siragusa
Pages:  203-210
To cite this article:  Blood Transfus 2010;8:203-10
Doi:  10.2450/2009.0101-09
Published online:  07/08/2009

Abstract

Introduction

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by thrombocytopenia, microangiopathic haemolytic anaemia, neurological and renal abnormalities and fever1, with a mortality rate, in the absence of treatment, of almost 90%. Since such criteria do not distinguish TTP from haemolytic uraemic syndrome (HUS), the comprehensive term TTP-HUS is more approriate2. The standard therapy is urgent plasma exchange (PE)1, which reduces mortality to 10% or less3-9. Because of its dramatic effect on short and long-term outcome, it is now accepted that PE can be begun, in the absence of an alternative diagnosis, even when not all of the above criteria are fulfilled3,4,6,9,10. The evident advantage of early initiation of therapy along with the decreased diagnostic threshold has resulted in a 7-fold increase of patients treated with PE for TTP-HUS from 1981 to 199711. [...]
  
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