Acute development of EBV+ T-cell lymphoproliferative disorder after treatment with rituximab for follicular lymphoma.

The sequential occurrence of two or more types of lymphomas is rare, particularly when they involve different cell lineages. We herein report a rare case of the sequential development of T-cell lymphoproliferative disorder (LPD) after treatment with rituximab for follicular lymphoma (FL). Although the efficacy and safety of rituximab have been established, the immunosuppressive effects of rituximab-containing therapy and the development of iatrogenic LPD/lymphoma, which is typically related to Epstein-Barr virus (EBV), need to be considered. EBV may infect T lymphocytes and manifest as hemophagocytic lymphohistiocytosis (HLH). Disease progression from HLH to clonal T-cell LPD has been reported in a high percentage of HLH patients. We herein report the clinical effects of HLH-2004 and CHOP in an elderly patient who developed EBV-positive T-cell LPD with HLH after treatment with rituximab for FL. An awareness of its clinical symptoms, bone marrow examination, and monitoring of the EBV load may help to discriminate EBV-positive T-cell LPD from the recurrence of FL or histologic transformation, and facilitate the timely initiation of life-saving therapies.