Characteristics of diffuse large B-cell lymphoma in patients with primary Sjogren's syndrome
Characteristics of diffuse large B-cell lymphoma in patients with primary Sjogren's syndrome
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DOI：
10.1111/1756-185x.13800
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发表时间：
2020-04-01
影响因子：
2.5
通讯作者：
Vasilyev, Vladimir Ivanovich
中科院分区：
文献类型：
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作者：
Gorodetskiy, Vadim Romanovich;Probatova, Natalya Alexandrovna;Vasilyev, Vladimir Ivanovich
目的原发性干燥综合征（pSS）患者发生弥漫性大B细胞淋巴瘤（DLBCL）的风险增加，DLBCL是一种侵袭性、异质性非霍奇金淋巴瘤。本研究的目的是表征DLBCL患者pSS.Method，我们确定了18例DLBCL和pSS超过22年的时间。根据2016年WHO指南，我们使用广泛的抗体进行免疫组化检测，并使用原位杂交进行EB病毒（EBV）检测，以确定DLBCL的特征。结果从最初的pSS症状发作到DLBCL诊断的中位时间为20.5年，从pSS诊断到DLBCL诊断的中位时间为14年。淋巴瘤诊断后，中位总生存期为3个月（范围：0-212个月），5年总生存率为37.5%。13例DLBCL重新分类为DLBCL，9例未另行说明（NOS）; 2例EBV阳性DLBCL，NOS; 2例富含T细胞/组织细胞的大B细胞淋巴瘤。5例DLBCL病例未重新分类，因为其EBV状态未知。结论pSS患者的DLBCL发生较晚，主要与DLBCL的非GCB亚型有关。
Aim Patients with primary Sjogren's syndrome (pSS) have an increased risk of developing diffuse large B-cell lymphoma (DLBCL), which is an aggressive and heterogeneous non-Hodgkin lymphoma. This study aimed to characterize DLBCLs in patients with pSS.Method We identified 18 patients with DLBCL and pSS over a 22-year period. Based on the 2016 WHO guidelines, we characterized DLBCL based on immunohistochemical tests using a broad panel of antibodies, and an Epstein-Barr virus (EBV) test using in situ hybridization.Results The median time from initial pSS symptom onset to the DLBCL diagnosis was 20.5 years and the median time from the pSS diagnosis until the DLBCL diagnosis was 14 years. After the lymphoma diagnosis, the median overall survival was 3 months (range: 0-212 months) and the 5-year overall survival rate was 37.5%. Thirteen DLBCLs were re-classified as DLBCL, not otherwise specified (NOS) in nine cases; EBV-positive DLBCL, NOS in two cases; and T-cell/histiocyte-rich large B-cell lymphoma in two cases. Five cases of DLBCLs were not re-classified because their EBV status was unknown. The Hans algorithm, which uses a combination of staining for CD10, BCL6, and MUM1, was used to classify the DLBCLs into the germinal center B-cell (GCB) subtype for three cases and the non-GCB subtype for nine cases.Conclusion These results indicate that DLBCL tends to occur late in pSS cases and is mainly related to the non-GCB subtype of DLBCL.