Rituximab for the treatment of autoimmune cytopenias in children with immune deficiency

Autoimmune cytopenias are well recognised in patients with primary immune deficiency, but treatment may be difficult. We report eight children with autoimmune cytopenias (autoimmune haemolytic anaemia, autoimmune thrombocytopenia, autoimmune neutropenia) complicating immune deficiency states (common variable immunodeficiency, Wiskott-Aldrich Syndrome, autoimmune lymphoproliferative syndrome, combined immunodeficiency) treated with between 1 and 3 courses of rituximab (anti-CD20). Responses occurred for 90% of treatments but relapse rates (after a median of 53 weeks) were high (78%). We conclude that rituximab is an effective treatment for autoimmune cytopenias in children with immune deficiencies, but repeated courses of treatment may be needed.