淋巴瘤合并干燥综合征或未分化结缔组织病的临床特征

Clinical difference between lymphoma and lymphoma with Sjogren's syndrome or undifferentiated connective tissue disease

目的探讨淋巴瘤合并干燥综合征(SS)或未分化结缔组织病(UCTD)的临床特征。方法回顾性分析2010年1月~2019年12月四川大学华西医院收治的淋巴瘤住院病例31187例,其中在出现淋巴瘤前曾诊断SS或UCTD的213例患者纳入A组,未出现免疫异常的淋巴瘤患者30974例纳入B组。比较两组患者发病年龄、性别、病理亚型、凝血指标[凝血酶原时间(PT)、活化部分凝血活酶时间(APTT)、纤维蛋白原(FIB)]、补体(C3、C4)、免疫球蛋白(IgA、IgG、IgM)、流式细胞学检测CD3、CD19、CD20以及生存率之间是否存在差异。结果A组发病年龄、女性比例均显著高于B组(P<0.05)。A、B组均以非霍奇金淋巴瘤为主,弥漫大B细胞淋巴瘤最多,A组中依次为黏膜相关阻滞细胞型淋巴瘤(34.4%)、滤泡型淋巴瘤(7.7%),B组中依次为滤泡淋巴瘤(14.4%)、黏膜相关阻滞细胞型淋巴瘤(9.1%)。A组PT、APTT、FIB值显著高于B组(P<0.05)。A、B组治疗前补体C3较B组降低明显,IgM水平均显著低于B组,IgG水平显著高于B组(P<0.05);A组以CD5阳性表达率最高,其次为CD20、CD3,CD19的阳性表达率最低,B组以CD20阳性表达率最高,其次为CD3和CD19,CD5的阳性表达率最低。A组患者生存率均显著高于B组(P<0.05)。结论对于年龄偏大、出现补体C3水平下降、IgG水平上升和IgM水平下降显著的女性淋巴瘤患者,应当注重对自身免疫性疾病的筛查,重视其高凝状态,同时治疗上应加强免疫调节治疗改善免疫紊乱状态,延缓疾病进展。

Objective To analyze the clinical difference between lymphoma and lymphoma with Sjogren's syndrome or undifferentiated connective tissue disease.Methods The clinical data of 31187 lymphoma patients who were initially treated in West China Hospital,Sichuan University from Jan.2010 to Dec.2019 were retrospectively analyzed.The 213 patients who diagnosed Sjogren's syndrome or undifferentiated connective tissue disease before lymphoma were included in group A,and 30974 other cases with lymphoma were included in group B.The differences of age,gender,lymphoma subtype,coagulation index,complement,immunoglobulin,immunophenotype and survival rate between the two groups were compared.Results The onset age of group A was significantly higher than that of group B(P<0.05).The proportion of women in group A was significantly higher than that in group B(P<0.05).Group A and B were mainly non Hodgkin's lymphoma,Diffuse large B-cell lymphoma is the most common.In group A,there were mucosa associated blocking cell lymphoma(34.4%)and follicular lymphoma(7.7%).In group B,follicular lymphoma(14.4%),mucosa associated blocking cell lymphoma(9.1%).The levels of PT,APTT and FIB in group A were significantly higher than those in group B(P<0.05).The levels of complement C3 and IgM before treatment in group A and B were significantly lower than those in group B,and the level of IgG in group A was significantly higher than that in group B(P<0.05).The decrease proportion of complement C3,the increase proportion of IgG and the decrease proportion of IgM in group A were significantly higher than those in group B(P<0.05).In group A,the positive expression rate of CD5 was the highest,followed by CD20,CD3,the lowest was CD19.In group B,the positive expression rate of C20 was the highest,followed by CD3 and CD19,and the lowest was CD5.The survival rate of group A were significantly higher than those of group B(P<0.05).Conclusion For female lymphoma patients with older age,decreased complement C3 level,increased IgG level and decreased IgM level,more attention should be paid in screening of autoimmune diseases and hypercoagulable state.Meanwhile,immunomodulatory therapy should be strengthened to improve the immune disorder and delay the progress of the disease.