干燥综合征和类风湿关节炎患者腮腺中复合型爱泼斯坦-巴尔病毒阳性黏膜相关淋巴组织淋巴瘤及爱泼斯坦-巴尔病毒阴性弥漫性大B细胞淋巴瘤：一例报告

Composite Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma and Epstein-Barr virus-negative diffuse large B-cell lymphoma in the parotid salivary gland of a patient with Sjögren's syndrome and rheumatoid arthritis: a case report.

作者信息

Gorodetskiy Vadim R, Probatova Natalya A, Konovalov Dmitry M, Ryzhikova Natalya V, Sidorova Yulia V, Sudarikov Andrey B, Mukhortova Olga V

机构信息

Department of Intensive Methods of Therapy, V.A. Nasonova Research Institute of Rheumatology, Kashirskoye shosse 34А, Moscow, 115522, Russia.

Department of Pathology, N.N. Blokhin Russian Cancer Research Center, Kashirskoye shosse 24, Moscow, 115478, Russia.

出版信息

J Med Case Rep. 2020 Jan 17;14(1):12. doi: 10.1186/s13256-019-2331-1.

DOI:10.1186/s13256-019-2331-1

PMID:31948469

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6966905/

Abstract

BACKGROUND

Epstein-Barr virus is associated with many human hematopoietic neoplasms; however, Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma is extremely rare. In routine clinical practice, detection of mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma in a tissue sample presumes a clonal relation between these neoplasms and that diffuse large B-cell lymphoma developed by transformation of the mucosa-associated lymphoid tissue lymphoma. However, evidence to support this presumption is sparse and controversial. Assessment of the clonal relationship of the lymphoid components of a composite lymphoma is important for understanding its pathogenesis and correct diagnosis.

CASE PRESENTATION

We present an unusual case of composite lymphoma (Epstein-Barr virus-positive mucosa-associated lymphoid tissue lymphoma/Epstein-Barr virus-negative diffuse large B-cell lymphoma) in the parotid salivary gland of a 62-year-old Caucasian woman with Sjögren's syndrome and rheumatoid arthritis. Simultaneous occurrence of mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma in the parotid salivary gland led us to initially assume a clonal relationship between diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue lymphoma. Epstein-Barr virus was detected by in situ hybridization and polymerase chain reaction in the mucosa-associated lymphoid tissue lymphoma, but not in diffuse large B-cell lymphoma, suggesting that these lymphomas were not clonally related. Fragment analysis of frame region 3 polymerase chain reaction products from microdissected mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma components revealed different clonal pattern rearrangements of the immunoglobulin heavy chain gene.

CONCLUSIONS

Our patient's case highlights the importance of assessing the clonal relationships of the lymphoid components of a composite lymphoma and Epstein-Barr virus screening in mucosa-associated lymphoid tissue lymphoma in patients with autoimmune disease.

摘要

背景

爱泼斯坦-巴尔病毒与许多人类造血系统肿瘤相关；然而，爱泼斯坦-巴尔病毒阳性的黏膜相关淋巴组织淋巴瘤极为罕见。在常规临床实践中，在组织样本中检测黏膜相关淋巴组织淋巴瘤和弥漫性大B细胞淋巴瘤时，假定这些肿瘤之间存在克隆关系，即弥漫性大B细胞淋巴瘤是由黏膜相关淋巴组织淋巴瘤转化而来。然而，支持这一假定的证据稀少且存在争议。评估复合淋巴瘤淋巴成分的克隆关系对于理解其发病机制和正确诊断至关重要。

病例报告

我们报告了一例不寻常的复合淋巴瘤（爱泼斯坦-巴尔病毒阳性的黏膜相关淋巴组织淋巴瘤/爱泼斯坦-巴尔病毒阴性的弥漫性大B细胞淋巴瘤），发生于一名患有干燥综合征和类风湿关节炎的62岁白种女性的腮腺。腮腺中同时出现黏膜相关淋巴组织淋巴瘤和弥漫性大B细胞淋巴瘤，这使我们最初假定弥漫性大B细胞淋巴瘤与黏膜相关淋巴组织淋巴瘤之间存在克隆关系。通过原位杂交和聚合酶链反应在黏膜相关淋巴组织淋巴瘤中检测到了爱泼斯坦-巴尔病毒，但在弥漫性大B细胞淋巴瘤中未检测到，这表明这些淋巴瘤并非克隆相关。对显微切割的黏膜相关淋巴组织淋巴瘤和弥漫性大B细胞淋巴瘤成分的框架区域3聚合酶链反应产物进行片段分析，揭示了免疫球蛋白重链基因不同的克隆模式重排。