Systemic lupus erythematosus (SLE) patients tends to have a higher risk of developing lymphoid malignancies. The majority of such tumors are of a B cell origin. However, it is known that the T cell lymphoma subtypes in SLE patients are quite rare. Here, we describe a case of peripheral T cell lymphoma, unspecified (PTCL-U) that occurred in a 50-year-old female SLE patient. The lymphoma was located at the bilateral cervical and mediastinal lymph nodes. The staging workup revealed no evidence of any other organ involvement. Epstein-Barr virus messenger RNA was detected in the serum, but not in the lymph nodes. She received front-line chemotherapy with the CHOP regimen and she achieved complete remission. She then subsequently received high-dose chemotherapy with autologous peripheral stem cell transplantation. The patient currently remains in a clinical and serological state of remission for the SLE and PTCL until the time of this report 18 months after chemotherapy, and this was followed by autologous peripheral blood stem cell transplantation. (Korean J Hematol 2009;44:273-277.)

Keywords: Lymphoma, T cell, Peripheral, Lupus erythematosus, Systemic, Peripheral blood stem cell transplantation