Haematologica
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Haematologica, Vol 92, Issue 12, 1691-1694 doi:10.3324/haematol.11540
Copyright © 2007 by Ferrata Storti Foundation
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Autoimmune Cytopenias

Rituximab therapy for childhood Evans syndrome

Brigitte Bader-Meunier, Nathalie Aladjidi, Françoise Bellmann, Fabrice Monpoux, Brigitte Nelken, Alain Robert, Corinne Armari-Alla, Capucine Picard, Françoise Ledeist, Martine Munzer, Karima Yacouben, Yves Bertrand, Antoine Pariente, Arnaud Chaussé, Yves Perel, Guy Leverger

From Assistance Publique-Hôpitaux de Paris, Service d’Hématologie-Oncologie Pédiatrique, Hôpital Robert Debré, Paris, France (BB-M, KY); Service d’Hématologie-Oncologie Pédiatrique, Hôpital Pellegrin, Bordeaux, France (NA, ACYP); Assistance Publique-Hôpitaux de Paris Service d’Hématologie-Immunologie-Oncologie Pédiatrique, Hôpital Armand Trousseau, Paris, France (FB, GL); Service d’Hématologie-Immunologie-Oncologie-Rhumatologie Pédiatrique, Hôpital Archet II, Nice, France (FM); Service d’Hématologie-Oncologie Pédiatrique, Hôpital Jeanne de Flandre, Lille, France (BN); Service d’Hématologie-Oncologie Pédiatrique, Hôpital des Enfants, Toulouse, France (AR); Service d’Hématologie-Oncologie Pédiatrique, Hôpital de La Tronche, La Tronche, France (CA-A); Assistance Publique-Hôpitaux de Paris, Centre d’étude des déficits immunitaires, Hôpital Necker-Enfants Malades, Paris, France (CP); Département de Microbiologie et Immunologie, CHU Sainte Justine , Université de Montréal, Canada (FL); Unité d’Onco-Hématologie Pédiatrique, American Memorial Hospital, Reims, France (MM); Unité d’Onco-Hématologie Pédiatrique, Hôpital Debrousse, Lyon, France (YB); INSERM U657, Hôpital Pellegrin, Département de pharmacologie, Université Bordeaux 2, Bordeaux, France (AP)

Correspondence: Brigitte Bader-Meunier, Service d’Hématologie Pédiatrique, Hôpital Robert Debré, 38 Bd Serrurier, 75019 Paris, France. E-mail: brigitte.badermeunier{at}rdb.ap-hop-paris.fr

The safety and efficacy of rituximab have been retrospectively assessed in 17 children with Evans syndrome. Patients received 4 or 3 weekly doses of rituximab (375 mg/m2 per dose) associated with prednisone, alone (14 patients) or associated with other immunosuppressive drugs. Complete or partial remission of at least one cytopenia was achieved in 13 out of the 17 patients (76%), and lasted in 11 of them with a mean follow-up of 2.4 years (range 0.5–7 years). Steroid therapy was stopped or tapered at 50–100% of the baseline dosage in all long-term responders. Moderate side effects and infection occurred only in 4 and 1 children respectively.

Key words: Evans syndrome, children, autoimmune hemolytic anemia, autoimmune thrombocytopenic purpura, rituximab.


Related Article

Rituximab for the treatment of autoimmune cytopenias
Sigbjørn Berentsen
Haematologica 2007 92: 1589-1596. [Full Text] [PDF]





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Copyright © 2007 by the Ferrata Storti Foundation.