【作者】 贾宁；

【导师】 唐福林；

【作者基本信息】 中国协和医科大学 ， 免疫内科， 2008， 硕士

【摘要】 目的分析原发性干燥综合征合(pSS)合并非霍奇金淋巴瘤(NHL)患者的临床表现、实验室血清学检查以及淋巴瘤组织学的特征。方法检索1982年10月至2008年10月我院住院患者数据库中pSS合并NHL的病例。分别记录pSS合并NHL主要的临床和实验室特点。结果共9例患者入组,其中女性8例(8/9,89%),男性1例(1/9,11%)。pSS和NHL诊断时平均年龄分别为50.6岁和55.6岁。8例(8/9,89%)符合美欧合议的干燥综合征分类标准(AECC)。患者主要的SS临床表现是无痛性腮腺肿大(7/9,78%),其中6例(6/9,67%)为单侧肿大;血清免疫学主要表现为类风湿因子(7/7,100%)阳性,滴度平均增高14.4倍(8.0倍～22.7倍),以及高免疫球蛋白血症(7/9,78%),其中IgG均增高(7/9,78%),平均增高69.4%(5.6%～215.6%),IgA增高3例(3/9,33%),平均增高95.5%(52.2%～176.3%),IgM增高2例(2/9,22%),平均增高221.8%(112.5%～331.0%);5例(5/9,56%)接受过糖皮质激素治疗,其中的4例(4/5,80%)还接受过至少一种免疫抑制剂(甲氨蝶呤、雷公藤多甙、环磷酰胺和依木兰)治疗。NHL主要临床表现包括脾脏肿大(7/9,78%)和淋巴结肿大(5/9,56%)。其主要病理组织亚型是黏膜相关淋巴组织(MALT)B细胞淋巴瘤(4/9,44%)和弥漫大B细胞淋巴瘤(2/9,22%)。MALT患者均无结区受累。8例(8/9,89%)患者出现结外受累,其中以累及涎腺(4/9,44%)和肺脏(4/9,44%)为最多。结论pSS合并NHL患者临床上以单侧腮腺(无痛性)、淋巴结和脾脏肿大,血清免疫学检查RF高滴度阳性和高球蛋白血症,MALT为主的NHL病理分型和高发结外受累为主要特征。更多还原

【Abstract】 Objective To characterize clinical patterns of expression, laboratory serologics, and lymphomatous histologics in patients with primary sj(o|¨)gren’s syndrome(pSS) who subsequently developed non-Hodgkin’s lymphoma(NHL).Metholds Searching in hospitalized patients database of our hospital admitted from October 1982 to October 2008. Identifying patients with pSS who developed NHL. A protocol form was used to record the main characteristics of pSS and NHL. Results Nine patients with pSS and NHL were included in the study. There were 8 (8/9, 89%) women and 1 (1/9, 11%) man (mean age 50.6 and 55.6 years at pSS and lymphoma diagnosis, respectively). Eight patients (8/9, 89%)fulfilled the American-European Consensus Criteria (AECC). The main SS manifestations were painless parotid enlargement (7/9, 78%), of whom six patients (6/9,67%) were unilateral; the main immunologic features were positive rheumatoid factor (RF) in all examined patients (7/7, 100%) with an average 14.4-fold (range 8.0-22.7) increased titre and hyperimmunoglobulinemia (7/9, 78%) with an average 69.4% (range 5.6%-215.6%) and 95.5% (range 52.2%-176.3%) and 221.8% (range 112.5%-331.0%) increased value of IgG (7/9, 78%) and IgA(3/9,33%) and IgM (2/9,22%) respectively; Five patients (5/9, 56%) had received glucocorticoids, of whom four patients (4/5,80%) had received at least one immunosuppressive drugs(methotrexate, glucosidorum tripterygll totorum, cyclophosphamide, and imuran). The main NHL manifestations were splenomegaly (7/9, 78%) and lymphadenopathy (5/9, 56%). The main histologic subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma (4/9, 44%) and diffuse large B cell lymphoma (2/9, 22%). None of the patients with MALT lymphoma had a nodal primary location. Eight patients (8/9, 89%) had an extranodal primary location, most frequently in the salivary gland (4/9, 44%) and lung (4/9, 44%). Conclusion Patients with pSS and NHL are clinically characterized by a high frequency of painless unilateral parotid enlargement and splenomegaly and lymphadenopathy, an immunologic pattern dominated by the presence of high-titre RF and hyperimmunoglobulinemia, a predominance of MALT lymphomas, and an elevated frequency of primary extranodal involvement.更多还原