Bonilla-Abadía Fabio, Pérez Manuel A, Muñoz-Buitrón Evelyn, Rosales Joaquín D, Cañas Carlos A, Tobón Gabriel J
Rheumatology Unit, Fundación Valle del Lili, ICESI University, Cali, Colombia.
Case Rep Rheumatol. 2013;2013:831708. doi: 10.1155/2013/831708. Epub 2013 Apr 23.
Primary Sjögren's syndrome (pSS) is an autoimmune disorder of the exocrine glands presenting with progressive ocular and oral dryness, parotid gland enlargement, and often with extraglandular manifestations. In this group of patients the risk of development of non-Hodgkin's lymphoma is 16-fold compared to healthy population, mainly of the MALT lymphoma type. This case reports a 52-year-old woman with pSS developing a progressively growing mass at face and neck compatible with blastoid variant mantle cell lymphoma and a fatal outcome.
原发性干燥综合征(pSS)是一种外分泌腺的自身免疫性疾病,表现为进行性眼干和口干、腮腺肿大,且常伴有腺体外表现。在这组患者中,非霍奇金淋巴瘤的发生风险是健康人群的16倍,主要为黏膜相关淋巴组织(MALT)淋巴瘤类型。本病例报告了一名52岁患有原发性干燥综合征的女性,其面部和颈部出现逐渐增大的肿块,符合母细胞样变异型套细胞淋巴瘤,最终导致死亡。