Haematologica (Dec 2007)

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

DOI
https://doi.org/10.3324/haematol.11540
Journal volume & issue
Vol. 92, no. 12

Abstract

Read online

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.