原发性干燥综合征合并淋巴瘤的临床研究

Characteristics of the Patients with Primary Sjgren's Syndrome Complicated with Lymphoma

目的探讨原发性干燥综合征(primary Sjgren’s syndrome,pSS)合并淋巴瘤患者的临床特点,治疗及其预后,以提高其诊治水平。方法回顾性分析2006年1月至2011年1月住院治疗的pSS合并淋巴瘤患者的临床特点、实验室检查、治疗和随访资料。结果确诊pSS合并淋巴瘤患者12例,占同期新诊断淋巴瘤的1.29%。患者pSS的特征性免疫学改变为高免疫球蛋白血症、低补体血症和CD4T细胞减少。病理类型以非霍奇金淋巴瘤(non-Hodgkin’s lymphoma,NHL)为主,其中弥漫大B细胞淋巴瘤最多。多数患者临床分期为Ⅲ～Ⅳ期,结外受累较常见。所有患者均接受联合化疗,NHL以CHOP/R-CHOP样方案化疗为主,霍奇金淋巴瘤(HL)以AVD方案化疗。中位随访时间27月(1～56月)。补体C3和C4共同下降的患者与非共同下降患者的中位生存时间、总体生存率(overallsurvival,OS)差异无统计学意义(P>0.05)。利妥昔单抗(rituximab)联合化疗组患者与不含rituximab的联合化疗组的中位生存时间、OS差异无统计学意义(P>0.05)。结论低补体血症不能认定为pSS合并淋巴瘤预后的影响因素。pSS合并淋巴瘤的预后相对不佳,含rituximab的联合化疗没有改善患者的疗效和生存。

Objective To improve the understanding of diagnosis and treatment of patients with primary Sjgren＇s syndrome（pSS） complicated with lymphoma.Methods The data of clinical features,laboratory findings,therapeutic response and follow-up of patients with primary Sjgren＇s syndrome complicated with lymphoma from January 2006 to January 2011 in our single center were retrospectively analyzed.Results Totally twelve inpatients with pSS complicated with lymphoma were diagnosed,which accounted for 1.29% of newly-diagnosed lymphoma inpatients during the same period.The characteristic immunologic changes were hyperimmunoglobulinemia,hypocomplementemia and decrease of CD4 T cell number.In our study,non-Hodgkin＇s lymphoma（NHL） was the most common type,and the main pathological subtype was diffuse large B cell lymphoma（DLBCL）.Most of the patients were in advanced stages,Ann Arbor stage Ⅲ-Ⅳ,at diagnosis.Extranodal involvement was common,most frequently in the livers and the lungs.All of the patients received combination chemotherapy.Most of the NHL patients received CHOP/R-CHOP-like regimens,and the Hodgkin＇s lymphoma（HL） patient received AVD regimen.The median follow-up time was 27 months（range 1～56 months）.In terms of median survival time and overall survival there were no statistical significant differences between both low C3 and low C4 group and control group（P0.05）.In terms of median survival time and overall survival there were no statistical significant differences between rituximab treatment group and control group（P0.05）.Conclusion The patients with pSS complicated with lymphoma were not uncommon clinically.Hypocomplementemia could not be identified as a risk factor for the prognosis of pSS complicated with lymphoma in our study.Although expected prognosis of these patients was unfavorable,we found that treatment with rituximab combination chemotherapy could not improve the therapeutic effects and survival of patients with pSS complicated with lymphoma.