Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece.
Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece.
Autoimmun Rev. 2015 Jul;14(7):641-9. doi: 10.1016/j.autrev.2015.03.004. Epub 2015 Mar 22.
The intrinsic and complex nature of primary Sjӧgren's syndrome (pSS) makes it difficult to identify risk factors that can predict the development and outcome of non-Hodgkin's lymphoma (NHL), yet patients at high risk for such complication seem to bear certain clinic-serological characteristics that render them a unique profile. In the last decade, research focusing on B-cell hyperactivity as the hallmark of pSS-related lymphoproliferation has shed light on certain biological and molecular factors that participate in disease evolution and lymphoma development, thus indicating possible predictors of lymphoma development and outcome. In this review, we explore all the available data concerning the clinical picture, risk prognostication and outcome of pSS-associated NHLs.
原发性干燥综合征（pSS）的内在和复杂性质使得难以确定可以预测非霍奇金淋巴瘤（NHL）发展和结局的风险因素，然而，存在此类并发症高风险的患者似乎具有某些临床血清学特征，使他们具有独特的特征。在过去十年中，专注于 B 细胞过度活跃作为 pSS 相关淋巴增生标志的研究揭示了参与疾病演变和淋巴瘤发展的某些生物学和分子因素，从而表明淋巴瘤发展和结局的可能预测因子。在这篇综述中，我们探讨了所有关于 pSS 相关 NHL 的临床表现、风险预测和结局的可用数据。
