Relapsing or refractory idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: the role of rituximab.
Transfusion
; 50(12): 2753-60, 2010 Dec.
Article
em En
| MEDLINE
| ID: mdl-20576013
ABSTRACT
Idiopathic thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is a rare disease responsive to treatment with plasma exchange (PE) but with a high percentage of relapse or refractory patients. A severe deficiency of ADAMTS-13 (<5% of normal activity), congenital or caused by an autoantibody, may be specific for TTP and it has been proposed that severe ADAMTS-13 deficiency now defines TTP. B cells play a key role in both the development and the perpetuation of autoimmunity, suggesting that B-cell depletion could be a valuable treatment approach for patients with idiopathic TTP-HUS. This review of the literature focuses on the role of rituximab, a chimeric monoclonal antibody directed against CD20 antigen expressed by B lymphocytes, in patients with relapsing or refractory TTP-HUS with or without ADAMTS-13 deficiency, suggesting that rituximab may produce clinical remission in a significant proportion of patients. Rituximab therapy reduces plasma requirement and avoids complications related to salvage-immunosuppressive therapy. In conclusion, rituximab provides an effective, well-tolerated, and safe treatment option for patients with idiopathic TTP-HUS, thus giving an alternative approach to the current treatment based on PE.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Púrpura Trombocitopênica Trombótica
/
Resistência a Medicamentos
/
Anticorpos Monoclonais Murinos
Tipo de estudo:
Evaluation_studies
Limite:
Humans
Idioma:
En
Revista:
Transfusion
Ano de publicação:
2010
Tipo de documento:
Article
País de afiliação:
Itália