Successful Rituximab Monotherapy in Advanced-stage, Epstein-Barr Virus-positive Diffuse Large B-Cell Lymphoma in an Adolescent With Systemic Juvenile Idiopathic Arthritis.

Epstein-Barr virus-positive diffuse large B-cell lymphoma (EBV+ DLBCL) in pediatrics most commonly occurs as an iatrogenic immunodeficiency-associated lymphoproliferative disease. We report an 18-year-old female individual with refractory systemic juvenile idiopathic arthritis, treated with multiple immunosuppressive agents, who was diagnosed with stage III, EBV+ DLBCL. The patient achieved sustained complete remission after 4 weekly doses of rituximab monotherapy and reduction of immunosuppression. This case suggests that a post-transplant lymphoproliferative disease-like treatment approach can be a safe and effective therapy in a nontransplant, yet severely immunosuppressed, patient with EBV+ DLBCL.