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Population-based study of patients with primary Sjögren's syndrome and lymphoma: lymphoma subtypes, clinical characteristics, and gender differences.

基于人群的原发性干燥综合征和淋巴瘤患者研究: 淋巴瘤亚型、临床特征和性别差异。

  • 影响因子:1.80
  • DOI:10.1080/03009742.2019.1696403
  • 作者列表:"Vasaitis L","Nordmark G","Theander E","Backlin C","Smedby KE","Askling J","Rönnblom L","Sundström C","Baecklund E
  • 发表时间:2020-05-01
Abstract

:Objective: To examine lymphoma subtypes, clinical characteristics, and gender differences in patients with primary Sjögren's syndrome (pSS) and lymphoma in a population-based setting.Method: 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.Results: 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).Conclusion: 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.

摘要

目的: 探讨原发性干燥综合征 (pSS) 和淋巴瘤患者的淋巴瘤亚型、临床特征和性别差异。方法: 诊断为干燥综合征和淋巴瘤的患者通过瑞典患者登记1964-2007与癌症登记1990-2007的联系来鉴定。从病历中收集临床数据,并重新检查淋巴瘤组织。结果: 我们确定了105例pSS淋巴瘤患者。弥漫大b细胞淋巴瘤 (DLBCL) (32%) 和边缘区淋巴瘤 [MZL包括粘膜相关淋巴组织 (MALT) 淋巴瘤] (31%) 是最常见的淋巴瘤亚型。与一般人群参照 (32%,p = 1) 相比,与MZL (一般人群5%,p <0.0001) 相比,DLBCL的比例没有增加。与DLBCL相比,MALT淋巴瘤的诊断年龄较小 (55 vs 67岁,p = 0.0001),在患者报告的sicca发作 (7 vs 18岁,p = 0.0001) 和pSS诊断 (2 vs 9岁,p = 0.0005) 后较早。16例pSS淋巴瘤病例为男性 (15%),是普通pSS人群的两倍。与女性相比,男性从pSS诊断到淋巴瘤诊断的中位时间较短 (1年vs 8年,p = 0.0003),并且唾液腺淋巴瘤更常见 (56% vs 29%,p = 0.04)。结论: DLBCL和MZL在pSS患者中很常见,但与一般人群相比,只有MZL/MALT淋巴瘤在pSS中发生的相对频率增加。该研究支持在pSS诊断后的第一年内提高对男性淋巴瘤体征的认识。

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影响因子:2.36
发表时间:2020-01-01
来源期刊:Platelets
DOI:10.1080/09537104.2019.1581922
作者列表:["Szanto T","Nummi V","Jouppila A","Brinkman HJM","Lassila R"]

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