Nodal EBV-positive polymorphic B cell lymphoproliferative disorder with plasma cell differentiation: clinicopathological analysis of five cases
Nodal EBV-positive polymorphic B cell lymphoproliferative disorder with plasma cell differentiation: clinicopathological analysis of five cases
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DOI：
10.1007/s00428-020-02967-6
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发表时间：
2020-11-09
期刊：
影响因子：
3.5
通讯作者：
Nakamura，Shigeo
中科院分区：
文献类型：
--
作者：
Satou，Akira;Tabata，Tetsuya;Nakamura，Shigeo
浆细胞分化（PCD）是常见的非霍奇金B细胞淋巴瘤的一些实体，包括低级别和高级别淋巴瘤。然而，除了浆母细胞淋巴瘤和原发性渗出性淋巴瘤外，EBV+B细胞淋巴增殖性疾病（LPD）伴PCD由于其罕见性尚未得到很好的解决。我们对5例淋巴结EBV+多态性B细胞LPD伴PCD（PBLPD-PCD）进行了临床病理学检查，最初诊断为多态性EBV+弥漫性大B细胞淋巴瘤，未另作说明（DLBCL-NOS）伴PCD（n= 3）和MTX相关B细胞LPD（MTX相关B-LPD）（n= 2）。1例伴发脑病变，临床诊断为EB病毒相关脑炎。该患者接受阿糖腺苷治疗，脑病变和淋巴结EBV+PBLPD-PCD病变均消失。另1例以Mott细胞分化为特征。此病例为首例EBV+B细胞淋巴瘤或LPD伴Mott细胞分化的病例。2例类风湿关节炎患者出现的MTX相关B细胞LPD在停用MTX后自行消退，其中4例进行了TCRγ和IGHPCR分析。TCR γ重排2例，IGH无重排。另外两例患者的这些基因没有重排。我们的结论是，淋巴结EBV+PBLPD-PCD是罕见的，具有异质性特征。PCR分析表明，EBV+PBLPD-PCD可能仅具有TCR克隆性，而无IGH克隆性。考虑到肿瘤细胞上CD 20表达的部分或完全丧失，这一结果可能会对EBV+PBLPD-PCD的准确诊断造成混淆，病理学家需要注意这一现象，以避免误诊。
Plasma cell differentiation (PCD) is frequently observed in some entities of non-Hodgkin B cell lymphoma, including both low-grade and high-grade lymphomas. However, except for plasmablastic lymphoma and primary effusion lymphoma, EBV+B cell lymphoproliferative disorder (LPD) with PCD has not been well addressed due to its rarity. We clinicopathologically examined five cases of nodal EBV+polymorphic B cell LPD with PCD (PBLPD-PCD) initially diagnosed as polymorphic EBV+diffuse large B cell lymphoma, not otherwise specified (DLBCL-NOS) with PCD (n= 3) and methotrexate-associated B cell LPD (MTX-associated B-LPD) (n= 2). One case had a concomitant brain lesion which was clinically diagnosed as EBV-related encephalitis. This patient received therapy with vidarabine, and both the brain lesion and the nodal EBV+PBLPD-PCD lesions disappeared. Another case was characterized by Mott cell differentiation. This case was the first reported case of EBV+B cell lymphoma or LPD with Mott cell differentiation. The two cases of MTX-associated B cell LPD which arose in patients with rheumatoid arthritis spontaneously regressed after MTX cessation.TCRγandIGHPCR analysis was performed in four cases. Two cases hadTCRγrearrangements, but noIGHrearrangements. The other two cases had no rearrangements in these genes. We concluded that nodal EBV+PBLPD-PCD is rare, with heterogeneous characteristics. PCR analysis revealed that nodal EBV+PBLPD-PCD may have onlyTCRclonality and noIGHclonality. Considering the partial or complete loss of CD20 expression on the tumor cells, this result may be confusing for accurate diagnosis of EBV+PBLPD-PCD, and pathologists need to be aware of this phenomenon to avoid misdiagnosis.