摘要:
本研究旨在探讨自身免疫病合并非霍奇金淋巴瘤(NHL)的临床和病理特点.回顾性分析6例自身免疫痛合并NHL患者的临床和病理特点,并观察其治疗结果.结果表明,6例患者中自身免疫病为干燥综合征(Sjogren's syndrome,SS)2例、类风湿关节炎(rheumatoid arthritis,RA)2例、克隆氏病(Crohn'S disease,CD)和溃疡性结肠炎(ulcerative colitis,UC)各1例.结性和结外部位NHL各3例,在病理类型方面弥漫大B细胞淋巴瘤(DL-BCL)3例、结外NK/T细胞淋巴瘤(ENKL)2例和非特指型外周T细胞淋巴瘤1例.CD10、Bc1-6和MUMI的免疫组织化学检测显示,3例DLBCL均为非生发中心型.2例ENKL的EBER均阳性.应用CHOP方案化疗或加用累及野放疗后,5例病人获完全缓解,1例(例4)为原发耐药.NHL诊断后中位生存时间为3年.在针对NHL治疗后,1例SS病人病情好转,2例(UC和CD)病人病情稳定无变化.3例病A(2例RA和1例SS)仍需继续应用泼尼松等治疗以控制原发病症状.结论:NHL是自身免疫病的严重并发症之一,在病理类型中非生发中心型DLBCL发生率增高.CHOP或加用累及野放疗对自身免疫病合并的NHL有效,但对自身免疫病本身的疗效有限.%This study was aimed to investigate the clinical characteristics and treatment of patients with autoimmune disease combined with non-Hodgkin lymphoma(NHL). The clinical characteristics and pathologic patterns of 6 patients with NHL who concurrently suffered from autoimmune diseases were analysed retrospectively from aspects of clinical course, pathologic features, and therapy. Treatment outcomes for autoimmune diseases and NHL were observed. The results showed that 6 patients included 4 females and 2 males, range in age from 28 to 65 years with a median age of 56 years. The autoimmune diseases are Sjogren's syndrome ( SS, 2 cases), rheumatoid arthritis ( RA, 2 cases ), ulcerative colitis (UC,1 case) and Crohn's disease(CD,l case). The NHL diseases located not only in the lymph node (n =3) but also in extranodal sites (n = 3 ). Histologically, 3 cases were diffuse large B cell lymphoma (DLBCL), 2 cases were extranodal nasal NK/T lymphoma (ENKL) and 1 case was peripheral T cell lymphoma, not otherwise specified.Based on CD10, Bcl-6 and MUM1 expression patterns, all 3 DLBCL were classified as non-GC subtype. EBER positive tumor cells were detected in 2 case of ENKL. 5 patients achieved a complete remission ( 83% ) and 1 patient was primary drag-resistant after CHOP chemotherapy or involved radiotherapy. Median survival from the time of lymphoma diagnosis was 3 years. 1 patient showed clinical improvement of the SS symptoms. 2 patients (CD and UC)showed stable state of disease and 2 patients with RA and 1 patient with SS needed continuing treatment for their autoimmune diseaes after chemotherapy for NHL. It is concluded that the development of NHL is one of the most serious complications in patients with autoimmune diseases. There is an increased frequency of non-GC subtype DLBCL. CHOP combined with or without radiotherapy proves to be effective for autoimmune disease patients with aggressive NHL but ineffective for concurrent autoimmune diseases.