Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
Department of Pathology, N.N. Blokhin Russian Cancer Research Center, Moscow, Russia.
Int J Rheum Dis. 2020 Apr;23(4):540-548. doi: 10.1111/1756-185X.13800. Epub 2020 Feb 25.
Patients with primary Sjögren'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.
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.
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.
These results indicate that DLBCL tends to occur late in pSS cases and is mainly related to the non-GCB subtype of DLBCL.
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 是一种侵袭性和异质性的非霍奇金淋巴瘤。本研究旨在对 pSS 患者的 DLBCL 进行特征分析。
我们在 22 年期间确定了 18 例 pSS 合并 DLBCL 患者。根据 2016 年 WHO 指南，我们使用广泛的抗体免疫组化试验和原位杂交 EBV 检测，对 DLBCL 进行特征分析。
从最初的 pSS 症状出现到 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。在淋巴瘤诊断后，总生存期的中位数为 3 个月（范围：0-212 个月），5 年总生存率为 37.5%。13 例 DLBCL 中有 9 例被重新分类为未另作说明的弥漫性大 B 细胞淋巴瘤（NOS）；2 例为 EBV 阳性的弥漫性大 B 细胞淋巴瘤，NOS；2 例为 T 细胞/组织细胞丰富的大 B 细胞淋巴瘤。5 例 DLBCL 未重新分类是因为其 EBV 状态未知。使用 Hans 算法（一种结合 CD10、BCL6 和 MUM1 染色的方法）对 3 例 DLBCL 进行分类，将其归入生发中心 B 细胞（GCB）亚型，9 例归入非 GCB 亚型。
这些结果表明，DLBCL 在 pSS 患者中发生较晚，主要与非 GCB 亚型的 DLBCL 相关。
