Primary Sjögren's syndrome (PSS) is an autoimmune disease of the exocrine glands that is presented with progressive ocular and oral dryness, parotid enlargement and often with systemic (extraglandular) manifestations. In patients with PSS the risk of development of non-Hodgkin's lymphoma (NHL) is 44-fold compared to healthy population. The risk is associated with certain characteristics of the disease's course: recurrent changes in the parotid glands, lymph node enlargement, skin vasculitis, peripheral neuropathy, anemia and lymphopenia. According to the morphologic and phenotypic characteristics, B-cell low-grade lymphomas prevail. This communication presents a 65-year-old woman with PSS who developed a follicular type B-cell NHL 21 years after the autoimmune disease had been diagnosed. The analysis of our case and the literature review summarize the characteristics of the course of PSS, histologic variants, and evolution and prognosis of NHL in this kind of patients.

译文

原发性干燥综合征 (PSS) 是外分泌腺的一种自身免疫性疾病,表现为进行性眼和口腔干燥,腮腺肿大,通常具有全身 (腺外) 表现。与健康人群相比,PSS患者发生非霍奇金淋巴瘤 (NHL) 的风险是44倍。风险与疾病过程的某些特征有关: 腮腺的反复变化,淋巴结肿大,皮肤血管炎,周围神经病变,贫血和淋巴细胞减少。根据形态学和表型特征,以b细胞低度淋巴瘤为主。本通讯介绍了一名65岁的PSS妇女,该妇女在诊断出自身免疫性疾病21年后发展为滤泡型b细胞NHL。本例的分析和文献综述总结了此类患者PSS的病程特征,组织学变异以及NHL的演变和预后。

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