title: Successful autologous peripheral blood stem cell transplantation for a patient with primary adrenal lymphoma with hemophagocytic syndrome.
abstract: 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.
authors.affiliation: Chiba Aoba Municipal Hospital, Chiba City, Chiba, Japan. ekotan@sf7.so-net.ne.jp
authors.forename: Taeko
authors.initials: T
authors.lastname: Uehara
authors.forename: Akira
authors.initials: A
authors.lastname: Yokota
authors.forename: Masahiro
authors.initials: M
authors.lastname: Onoda
authors.forename: Kyohei
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Takashi
authors.initials: T
authors.lastname: Terano
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000473:pathology / Q000628:therapy*; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000473:pathology / Q000628:therapy*; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D036102:Peripheral Blood Stem Cell Transplantation; D011241:Prednisone / Q000008:administration & dosage; D012074:Remission Induction; D000069283:Rituximab; D014182:Transplantation, Autologous; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000069283:Rituximab; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone