Rituximab Monotherapy for EBV-related Lymphoproliferative Disease in Immunocompetent Boy

Abstract

We describe an EBV-related lymphoproliferative disorder in a 12-year-old boy who neither has evidence of recurrent infection nor family history of immunodeficiency disease. He had suffered from prolonged high fever, general fatigue and cervical lymphadenopathy for six months before admission. The EBV-related B cell lymphoproliferative disorder was confirmed by surgical open biopsy of the lymph nodes. Corticosteroid treatment was temporarily effective, and lymph node enlargement recurred in multiple areas. Although memory B cells disappeared from the patient’s peripheral blood 4 months after the initiation of steroid treatment, no hypogammaglobulinemia was observed during his clinical course. Rituximab monotherapy was initiated for this steroid-resistant and recurrent disease. After the administration of rituximab, the enlarged lymph nodes dramatically shrunk, and steroid administration was stopped immediately after rituximab therapy. No recurrence occurred for 24 months after rituximab therapy.