Rituximab therapy for childhood Evans syndrome.
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.
Bader-Meunier
Brigitte
B
Assistance Publique-Hôpitaux de Paris, Service d'Hématologie-Oncologie Pédiatrique, Hôpital Robert Debré, Paris, France. brigitte.badermeunier@rdb.ap-hop-paris.fr
Aladjidi
Nathalie
N
Bellmann
Françoise
F
Monpoux
Fabrice
F
Nelken
Brigitte
B
Robert
Alain
A
Armari-Alla
Corinne
C
Picard
Capucine
C
Ledeist
Françoise
F
Munzer
Martine
M
Yacouben
Karima
K
Bertrand
Yves
Y
Pariente
Antoine
A
Chaussé
Arnaud
A
Perel
Yves
Y
Leverger
Guy
G
D000293:Adolescent; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications / Q000188:drug therapy* / Q000401:mortality; D000911:Antibodies, Monoclonal / Q000008:administration & dosage* / Q000009:adverse effects; D058846:Antibodies, Monoclonal, Murine-Derived; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage* / Q000009:adverse effects; D007223:Infant; D007239:Infections / Q000139:chemically induced / Q000401:mortality; D008297:Male; D011239:Prednisolone / Q000008:administration & dosage / Q000009:adverse effects; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications / Q000188:drug therapy* / Q000401:mortality; D012042:Registries; D012074:Remission Induction; D000069283:Rituximab; D015996:Survival Rate; D013577:Syndrome
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab; D011239:Prednisolone