原发性干燥综合征合并非霍奇金淋巴瘤九例

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 9例pSS合并NHL的病例,其中女8例,男1例.5例接受过糖皮质激素治疗,其中的4例还接受过至少一种免疫抑制剂治疗.分析pSS合并NHL主要的临床和实验室特点.结果 8例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例),其中6例为单侧肿大;血清免疫学主要表现为类风湿因子(7例)阳性,以及高免疫球蛋白血症(7例).NHL主要临床表现包括脾脏肿大(7例)和淋巴结肿大(5例).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例)和弥漫大B细胞淋巴瘤(2例).MALT患者均无结区受累.8例患者出现结外受累,其中以累及涎腺(4例)和肺脏(4例)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,类风湿因子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.

Characteristics of patients with primary Sj(o)gren's syndrome and non-Hodgkin's lymphoma: analysis of 9 cases

Objective To characterize the clinical patterns of expression,laboratory serologic parameters and lymphomatous histological characteristics in patients with primary Sj(o)gren's syndrome (pSS) who subsequently developed non-Hodgkin's lymphoma (NHL).Methods The authors analyzed 9 pSS patients (8 females,1 male) who developed NHL.Five patients had received glucocorticoids,four of whom had received at least one immunosuppressive drags (methotrexate,glucosidorum tripterygll totornm,cyclophosphamide and imuran).A protocol form was used to record the main characteristics of pSS and NHL.Results Eight patients fulfilled the American-European Consensus Criteria (AECC).The main SS manifestations were painless parotid enlargement (n = 7),six of whom were unilateral;the main immunologic features were positive rheumatoid factor (RF) in all examined patients and hyperimmunoglobulinemia (n = 7).The main manifestations of NHL were splenomegaly (n = 7) and lymphadenopathy (n = 5).The main histological subtypes were mucosa-associated lymphoid tissue (MALT) lymphoma (n =4) and diffuse large B cell lymphoma (n =2).None of the patients with MALT lymphoma had a nodal primary location.Eight patients had an extranedal primary location,most frequently in salivary gland (n =4) and lung (n = 4).Conclusion Patients with pSS and NHL are clinically characterized by a high frequency of painless unilateral parotid enlargement,splenomegaly,lymphadenopathy,an immunologic pattern dominated by the presence of high-titer RF and hyperimmunoglobulinemia,a predominance of MALT lymphomas and an elevated frequency of primary extranodal involvement.