Acquired C1-inhibitor deficiency: 7 patients treated with rituximab.
J Clin Immunol
; 32(5): 936-41, 2012 Oct.
Article
en En
| MEDLINE
| ID: mdl-22526593
ABSTRACT
BACKGROUND:
Acquired C1-inhibitor deficiency can occur secondary to excessive C1-inhibitor consumption (type I) and be associated with a lymphoid hemopathy, or linked to the presence of anti-C1-inhibitor autoantibodies (type II) in a context of an isolated monoclonal gammopathy, sometimes associated with lymphoproliferation. Efficacy of danazol, tranexamic acid and/or corticosteroids is inconstant. Rituximab efficacy against type II angioedema has been reported.METHODS:
Description of 7 rituximab-treated patients, 6 with type II acquired angioedema and 1 with type I.RESULTS:
Clinical efficacy (only for type II) was complete for 3, partial for 2 and 2 were therapeutic failures. Only 2 patients had improved biological parameters, with normalization of their C1-inhibitor levels and diminished anti-C1-inhibitor autoantibodies, observed 1-9 months after the last infusion of the second rituximab cycle. An associated lymphoproliferation did not affect the response to treatment.CONCLUSION:
Rituximab efficacy in the treatment of acquired angioedema is inconstant and might require repeated cycles.
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Banco de datos:
MEDLINE
Asunto principal:
Proteínas Inactivadoras del Complemento 1
/
Anticuerpos Monoclonales de Origen Murino
/
Factores Inmunológicos
/
Angioedema
Límite:
Aged
/
Aged80
/
Female
/
Humans
/
Male
/
Middle aged
Idioma:
En
Año:
2012
Tipo del documento:
Article