[自身免疫性疾病合并非霍奇金淋巴瘤的临床病理特征]

Yu Ya-Ping, Liu Hai-Ning, Zhai Yong-Ping, Shi Ping, Song Ping, Li Feng, Zhou Xiao-Gang, Tang Yu-Mei

Department of Hematology, Nanjing General Hospital of Chinese PLA Nanjing Military Area, Nanjing 210002, Jiangsu Province, China.

Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2011 Feb;19(1):124-9.

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, 1 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 drug-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 diseases 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.

本研究旨在探讨自身免疫性疾病合并非霍奇金淋巴瘤（NHL）患者的临床特征及治疗方法。回顾性分析6例同时患有自身免疫性疾病的NHL患者的临床特征及病理类型，从临床病程、病理特征及治疗等方面进行分析，并观察自身免疫性疾病及NHL的治疗效果。结果显示，6例患者中4例为女性，2例为男性，年龄范围为28至65岁，中位年龄为56岁。自身免疫性疾病包括干燥综合征（SS，2例）、类风湿关节炎（RA，2例）、溃疡性结肠炎（UC，1例）和克罗恩病（CD，1例）。NHL病变不仅累及淋巴结（n = 3），也累及结外部位（n = 3）。组织学上，3例为弥漫大B细胞淋巴瘤（DLBCL），2例为结外鼻型NK/T淋巴瘤（ENKL），1例为未另行特指的外周T细胞淋巴瘤。根据CD10、Bcl-6和MUM1表达模式，所有3例DLBCL均被归类为非生发中心（non-GC）亚型。2例ENKL检测到EBER阳性肿瘤细胞。5例患者（83%）达到完全缓解，1例患者在CHOP化疗或局部放疗后原发耐药。自淋巴瘤诊断起的中位生存期为3年。1例患者SS症状有临床改善，2例患者（CD和UC）疾病状态稳定，2例RA患者和1例SS患者在NHL化疗后自身免疫性疾病仍需继续治疗。结论是，NHL的发生是自身免疫性疾病患者最严重的并发症之一。非GC亚型DLBCL的发生率增加。CHOP联合或不联合放疗对侵袭性NHL的自身免疫性疾病患者有效，但对同时存在的自身免疫性疾病无效。