The occurrence of diffuse large B-cell lymphoma (DLBCL) in the course of Sjogren's syndrome (SS) is considered to be equally related either to the development of DLBCL de novo or to the transformation from marginal zone lymphoma (MZL). However, the question of possible clonal relationship between MZL and DLBCL in the group of SS patients remains open. Here we present the data concerning 194 patients with lymphoma complicated SS followed up at Nasonova Research Institute of Rheumatology during the last 22 years. Molecular analysis of tumor cells was performed for 6 SS patients who had developed both MZL and DLBCL. To assess clonal relationship between each of the tumor pairs immunoglobulin heavy chain (IGH) gene rearrangements were identified according BIOMED-2 protocol by means of multiplex polymerase chain reaction followed by GeneScan fragment analysis. Despite different localization MZL and DLBCL were clonally related in five tumor pairs. The median time to transformation was 11 months (range 0-78 months). MZL and DLBCL were clonally related in most cases from our cohort of SS patients. No statistically significant difference in survival between patients with DLBCL transformed from MZL and patients with de novo DLBCL was found in the cohort of SS patients investigated.

中文翻译：

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Sjogren综合征患者边缘区淋巴瘤与弥漫性大B细胞淋巴瘤的克隆关系：病例系列研究和文献综述。

在干燥综合征（SS）的过程中，弥漫性大B细胞淋巴瘤（DLBCL）的发生被认为与DLBCL从头发展或边缘区淋巴瘤（MZL）的转化同等相关。但是，SS患者组中MZL和DLBCL之间可能存在克隆关系的问题仍然悬而未决。在这里，我们提供了有关Nasonova风湿病研究所过去22年中随访的194例并发SS淋巴瘤患者的数据。对6例同时患有MZL和DLBCL的SS患者进行了肿瘤细胞的分子分析。为了评估每个肿瘤对之间的克隆关系，根据BIOMED-2方案，通过多重聚合酶链反应，然后通过GeneScan片段分析，鉴定了免疫球蛋白重链（IGH）基因重排。尽管定位不同，但MZL和DLBCL在五个肿瘤对中克隆相关。转化的中位数时间为11个月（范围为0-78个月）。在我们的SS患者队列中，大多数情况下MZL和DLBCL是克隆相关的。在研究的S​​S患者队列中，从MZL转化的DLBCL患者与从头DLBCL患者之间的存活率在统计学上没有显着差异。在我们的SS患者队列中，大多数情况下MZL和DLBCL是克隆相关的。在研究的S​​S患者队列中，从MZL转化的DLBCL患者与从头DLBCL患者之间的存活率在统计学上没有显着差异。在我们的SS患者队列中，大多数情况下MZL和DLBCL是克隆相关的。在研究的S​​S患者队列中，从MZL转化的DLBCL患者与从头DLBCL患者之间的存活率在统计学上没有显着差异。