Unusual primary central nervous system T-cell/histiocyte-rich large B-cell lymphoma: a case report.

We report the case of a 54-year-old immunocompetent woman who presented with a primary T-cell/histiocyte-rich large B-cell lymphoma (TCHRLBCL) of the central nervous system without systemic involvement, diagnosed by means of a brain biopsy. She was treated with corticosteroids and we subsequently started chemotherapy with rituximab, methotrexate, ifosfamide and intrathecal cytarabine. The patient's symptoms gradually improved over the first weeks and we followed-up with autologous haematopoietic cell transplantation. The patient has been in complete remission for a year. Primary TCHRLBCL of the central nervous system in an immunocompetent patient is an extremely rare condition that requires a multidisciplinary approach. This case highlights the importance of undergoing a sequential work-up and establishing a treatment despite the absence of evidence-based guidelines.