Successful Autologous Peripheral Blood Stem Cell Transplantation for a Patient with Primary Adrenal Lymphoma with Hemophagocytic Syndrome

The adrenal glands are often an affected extranodal site in advanced non-Hodgkin lymphoma, but primary adrenal lymphoma (PAL) is extremely rare. Histologic examination of adrenal glands from patients with PAL reveals predominantly diffuse large B-cell lymphoma. Adrenal insufficiency is observed in about two thirds of patients. The prognosis of patients with PAL is poor: > 90% of patients die within 1 year of diagnosis, and long-term survivors are few. Effective therapeutic management of the condition has not been established. Herein, we report a 57-year-old man with PAL of diffuse large B-cell type that was later accompanied by hemophagocytic syndrome. He was initially treated with combination chemotherapy (1 cycle of CHOP [cyclophosphamide/doxorubicin/vincristine/prednisone] and 4 cycles of rituximab plus CHOP) and subsequently underwent autologous peripheral blood stem cell transplantation (auto-PBSCT) in his first complete remission. He has been well and disease free for 39 months since undergoing auto-PBSCT. The successful outcome of using auto-PBSCT in the treatment of a patient with PAL suggests that this therapeutic approach has value in treating this poor-prognostic disease.