系统性红斑狼疮合并淋巴瘤临床特征

摘要

目的 探讨系统性红斑狼疮(SLE)合并恶性淋巴瘤(ML)的临床特点.方法 回顾1998年1月至2012年2月北京协和医院收治的9例SLE合并ML患者的临床资料,分析其临床表现、实验室检查指标、淋巴瘤病理分型、治疗及预后.结果 SLE合并ML患者共9例,占同期SLE入院患者的0.18%;其中女性患者7例,男性患者2例;SLE平均发病年龄43.1岁(24～57岁),SLE平均确诊年龄为44.7岁(29～57岁),淋巴瘤平均确诊年龄为48.7岁(39～63岁),SLE发病到合并淋巴瘤病程均数为3.8年(1～15年).淋巴瘤病理类型以非霍奇金淋巴瘤(NHL)为主,共7例,占77.8%(7/9),且淋巴瘤结外受累多见,共7例,占77.8%(7/9).淋巴瘤在消化系统、呼吸系统、骨髓等部位均可发生,其中胃肠道受累3例,支气管和肺受累2例,骨髓受累2例.SLE合并ML患者中100%出现关节痛、淋巴结肿大及发热.实验室检查结果发现9例患者中6例补体减低,9例乳酸脱氢酶升高,5例红细胞沉降率(ESR)升高大于100mm/h.SLE合并ML的患者6例对化疗敏感,4例死亡,2例死因均为淋巴瘤消化道受累;2例诊断淋巴瘤放弃治疗后死亡.结论 当SLE患者出现不明原因持续发热、淋巴结肿大、ESR持续大于100mm/h,乳酸脱氢酶持续偏高者需警惕SLE合并淋巴瘤的可能.当SLE合并淋巴瘤时结外受累多见,SLE合并ML多数对化疗敏感,但消化道受累时预后差.%Objective To investigate the clinical characteristics of systemic lupus eryLhematosus ( SLE ) vviLh malignant lymphoma ( ML). Methods The clinical manifestations, laboratory tests, pathology, treatment and prognosis of SLE vviLh ML paLienLs from January 1998 to February 2012 in Peking Union Medical College Ilospital were reviewed and analyzed. Results Mine cases of SLE patients complicated vviLh ML were collecLed and Lhe prevalence of ML was 0. 18% of the total SLE patients during the same period. Among Lhem, 7 were women and 2 were men. The mean age aL disease onset and diagnosis of SLE was 43. 1 and 44. 7 years old respectively. The average disease course from disease onset of SLE Lo lymphoma was 3. 8 years ( 1-15 years ). The predominate paLhological type of lymphoma was non-Ilodgkin ' s lymphoma (NHL), which accounted for 77.8% (7/9). Digestive system was involved in 3 NHL the cases, respiratory system invotvement in 2 cases, and hone marrow invotvement in 2 cases. The ctinicat manifestations of StE compticated vvith Mt inctuded joint pain, tymph node entargement, fever, muscte pain and weakness, tiver and spteen entargement Att the six patients had joint pain, tymph node entargement and fever. taboratory tests indicated decreased comptement tevet in 6 cases; tactate dehydrogenase ( tD11 ) etevated in 9 cases, and btood sedimentau'on rate higher than 100 mm/h in 5 cases. Six patients of StE with Mt were sensitive to chemotherapy. Four cases died, in which 2 patients had digestive tract invotvement and the other 2 cases gave up treatment at diagnosis of tymphoma. Conctusion When StE patients experience tong-term fever without obvious causes, tymph node entargement tD11 etevation and btood sedimentation greater than 100 mm/h, attention shoutd be paid to the possibitity of tymphoma. Extracapsutar invotvement is common in StE combined vvith tymphoma. Akhough StE with tymphoma is sensitive to chemotherapy , the prognosis is poor when digestive tract is invotved.