Department of Medical Sciences, Section of Rheumatology, Uppsala University, Uppsala, Sweden.
Department of Rheumatology, Skåne University Hospital, Lund University, Malmö, Sweden.
Scand J Rheumatol. 2020 May;49(3):225-232. doi: 10.1080/03009742.2019.1696403. Epub 2020 Mar 10.
: To examine lymphoma subtypes, clinical characteristics, and gender differences in patients with primary Sjögren's syndrome (pSS) and lymphoma in a population-based setting.: Patients with Sjögren's syndrome and lymphoma diagnoses were identified by linkage of the Swedish Patient Register 1964-2007 with the Cancer Register 1990-2007. Clinical data were collected from medical records and lymphoma tissues were re-examined. The lymphoma subtype distribution was compared with the Swedish Lymphoma Register.: We identified 105 pSS patients with lymphoma. Diffuse large B-cell lymphoma (DLBCL) (32%) and marginal zone lymphoma [MZL including mucosa-associated lymphoid tissue (MALT) lymphoma] (31%) were the most common lymphoma subtypes. The proportion of DLBCL was not increased compared to the general population reference (32%, p = 1), in contrast to MZL (general population 5%, p < 0.0001). Compared to DLBCL, MALT lymphoma was diagnosed at a younger age (55 vs 67 years, p = 0.0001), and earlier after patient-reported sicca onset (7 vs 18 years, p = 0.0001) and pSS diagnosis (2 vs 9 years, p = 0.0005). Sixteen of the pSS-lymphoma cases were men (15%), twice the proportion in general pSS populations. Compared to women, men had a shorter median time from pSS diagnosis to lymphoma diagnosis (1 vs 8 years, p = 0.0003) and more often had lymphoma in the salivary glands (56% vs 29%, p = 0.04).: DLBCL and MZL are common in pSS patients, but only MZL/MALT lymphoma occurs at an increased relative frequency in pSS compared to the general population. The study supports increased awareness of signs of lymphoma in men in the first years after pSS diagnosis.
: 目的：在基于人群的环境中，研究原发性干燥综合征（pSS）和淋巴瘤患者的淋巴瘤亚型、临床特征和性别差异。
: 通过 1964-2007 年瑞典患者登记册与 1990-2007 年癌症登记册的链接，确定干燥综合征和淋巴瘤诊断的患者。从病历中收集临床数据，并重新检查淋巴瘤组织。将淋巴瘤亚型分布与瑞典淋巴瘤登记册进行比较。
: 我们确定了 105 例 pSS 伴淋巴瘤患者。弥漫性大 B 细胞淋巴瘤（DLBCL）（32%）和边缘区淋巴瘤[包括黏膜相关淋巴组织（MALT）淋巴瘤的 MZL]（31%）是最常见的淋巴瘤亚型。与一般人群参考相比，DLBCL 的比例并未增加（32%，p = 1），而 MZL 则增加（一般人群为 5%，p < 0.0001）。与 DLBCL 相比，MALT 淋巴瘤的诊断年龄较小（55 岁比 67 岁，p = 0.0001），且从患者报告的干燥症状发作后更早（7 年比 18 年，p = 0.0001）和干燥综合征诊断后更早（2 年比 9 年，p = 0.0005）。16 例 pSS-淋巴瘤病例为男性（15%），是一般 pSS 人群的两倍。与女性相比，男性从干燥综合征诊断到淋巴瘤诊断的中位时间更短（1 年比 8 年，p = 0.0003），且更常发生唾液腺淋巴瘤（56%比 29%，p = 0.04）。
: DLBCL 和 MZL 在 pSS 患者中很常见，但只有 MZL/MALT 淋巴瘤在 pSS 患者中的相对频率高于一般人群。该研究支持在 pSS 诊断后最初几年中，提高对男性淋巴瘤迹象的认识。
