Uehara Taeko, Yokota Akira, Onoda Masahiro, Yamamoto Kyohei, Terano Takashi
Chiba Aoba Municipal Hospital, Chiba City, Chiba, Japan.
Clin Lymphoma Myeloma. 2008 Jun;8(3):184-7. doi: 10.3816/CLM.2008.n.024.
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.
肾上腺常是晚期非霍奇金淋巴瘤的结外受累部位，但原发性肾上腺淋巴瘤（PAL）极为罕见。对PAL患者的肾上腺进行组织学检查显示，主要为弥漫性大B细胞淋巴瘤。约三分之二的患者存在肾上腺功能不全。PAL患者的预后较差：超过90%的患者在诊断后1年内死亡，长期存活者很少。目前尚未确立针对该疾病的有效治疗方案。在此，我们报告一名57岁男性，患有弥漫性大B细胞型PAL，随后并发噬血细胞综合征。他最初接受了联合化疗（1个周期的CHOP[环磷酰胺/阿霉素/长春新碱/泼尼松]以及4个周期的利妥昔单抗联合CHOP），随后在首次完全缓解期接受了自体外周血干细胞移植（auto-PBSCT）。自接受auto-PBSCT以来，他已健康无病39个月。在一名PAL患者中使用auto-PBSCT取得成功结果，表明这种治疗方法在治疗这种预后不良的疾病方面具有价值。
