Pediatric Rheumatology Online Journal → July 2003 → Childhood Lupus→ Treatment → Abstract #100
                                                                August 2003 → Newer Treatments → Anti-CD20 Monoclonal Antibody (Rituximab) → Abstract #100

ANTI-CD20 (RITUXIMAB) TREATMENT FOR REFRACTORY THROMBOCYTOPENIA IN JUVENILE SLE

R. Ten Cate,^1,2 F. J. Smiers,¹ R. G. Bredius,¹ A. C. Lankester,¹ M. van Veenendaal,¹ L. W. van Suijlekom-Smit,² R. M. Egeler.¹

¹Pediatrics, Leiden University Medical Center, Leiden, Netherlands; ²Pediatrics, Erasmus Medical Center / Sophia Children's Hospital, Rotterdam, Netherlands

A seven year old girl presented with polyarthritis, mouth ulcers, hemolytic anemia, thrombocytopenia, granulocytopenia in the presence of circulating antinuclear antibodies. She was diagnosed with SLE. Medical treatment consisted of naproxen, hydroxychloroquine and monthly infusions of gammaglobulins with good effect. At the age of ten years she had an episode with two convulsions with elevated intracranial pressure. Magnetic resonance imaging revealed a hypodense lesion in the occipital right lobe which disappeared after high dose corticosteroid treatment. At the age of twelve new cerebral lesions were detected and she was treated with pulse cyclophosphamide and methylprednisolon; for half a year monthly and for the following year three-monthly. Despite this treatment-regimen a new cerebral lesion evolved. For her perseverant deep thrombocytopenia and evolving granulocotypenia, splenectomy was performed at the age of thirteen. In the following months an autologous stem cell transplantation was prepared. This procedure was postponed because the cerebral lesions had decreased and the cytopenia had disappeared after splenectomy. One year later at age fifteen her plateletcount dropped again below 10x10⁹/L. Methylprednisolon pulse therapy and high dose intravenous gammaglobulins had no effect. Rituximab, an anti-CD20 monoclonal antibody was subsequently planned for treatment of this extremely recalcitrant thrombocytopenia. Four weekly doses of Rituximab (375 mg/m) were infused. No adverse reactions were seen. Soon after the first infusion a rise in platelets occurred. Since then her plateletcount remained above 400x10⁹/L, with a follow-up of 12 a year.
Use of anti-CD20, Rituximab has to be considered as treatment for life-threatening autoimmune cytopenias.