Rituximab therapy for childhood Evans syndrome

• Published in: Haematologica (Roma) Vol. 92; no. 12; pp. 1691 - 1694
• Main Authors: Bader-Meunier, Brigitte, Aladjidi, Nathalie, Bellmann, Francoise, Monpoux, Fabrice, Nelken, Brigitte, Robert, Alain, Armari-Alla, Corinne, Picard, Capucine, Ledeist, Francoise, Munzer, Martine, Yacouben, Karima, Bertrand, Yves, Pariente, Antoine, Chausse, Arnaud, Perel, Yves, Leverger, Guy
• Format: Journal Article
• Language: English
• Published: Pavia Haematologica 01.12.2007; Ferrata Storti Foundation
• Subjects: Adolescent; Anemia, Hemolytic, Autoimmune - complications; Anemia, Hemolytic, Autoimmune - drug therapy; Anemia, Hemolytic, Autoimmune - mortality; Anemias. Hemoglobinopathies; Antibodies, Monoclonal - administration & dosage; Antibodies, Monoclonal - adverse effects; Antibodies, Monoclonal, Murine-Derived; Biological and medical sciences; Child; Child, Preschool; Disease-Free Survival; Diseases of red blood cells; Female; Follow-Up Studies; France; Hematologic and hematopoietic diseases; Humans; Immunologic Factors - administration & dosage; Immunologic Factors - adverse effects; Infant; Infection - chemically induced; Infection - mortality; Male; Medical sciences; Platelet diseases and coagulopathies; Prednisolone - administration & dosage; Prednisolone - adverse effects; Purpura, Thrombocytopenic, Idiopathic - complications; Purpura, Thrombocytopenic, Idiopathic - drug therapy; Purpura, Thrombocytopenic, Idiopathic - mortality; Registries; Remission Induction; Rituximab; Survival Rate; Syndrome; France; Human; Autoimmunity; Immunopathology; Hematology; Rituximab; Autoimmune disease; Hemopathy; Monoclonal antibody; Evans syndrome; Platelet; Hemolytic anemia; Treatment; Autoimmune hemolytic anemia; children; Immunotherapy; Anticytokine; Immune thrombocytopenic purpura; Child; autoimmune thrombocytopenic purpura
• ISSN: 0390-6078; 1592-8721
• DOI: 10.3324/haematol.11540

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/m 2 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]