Human herpesvirus 8-negative effusion-based large B-cell lymphoma: a distinct entity with unique clinicopathologic characteristics.
Human herpesvirus 8-negative effusion-based large B-cell lymphoma: a distinct entity with unique clinicopathologic characteristics.
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DOI：
10.1038/s41379-022-01091-x
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发表时间：
2022-10
期刊：
影响因子：
7.5
通讯作者：
Pan, Zenggang
中科院分区：
文献类型：
--
作者：
Gisriel, Savanah D.;Yuan, Ji;Braunberger, Ryan C.;Maracaja, Danielle L., V;Chen, Xueyan;Wu, Xiaojun;McCracken, Jenna;Chen, Mingyi;Xie, Yi;Brown, Laura E.;Li, Peng;Zhou, Yi;Sethi, Tarsheen;McHenry, Austin;Hauser, Ronald G.;Paulson, Nathan;Tang, Haiming;Hsi, Eric D.;Wang, Endi;Zhang, Qian-Yun;Young, Ken H.;Xu, Mina L.;Pan, Zenggang
罕见的人类疱疹病毒8型（HHV 8）阴性渗出液型大B细胞淋巴瘤（EB-LBCL）发生在体腔内，没有先前或并发的实性肿块形成。与HHV 8+原发性渗出性淋巴瘤（PEL）相比，EB-LBCL与HIV或HHV 8感染无已知相关性。然而，全球病例报告和系列的样本量较小，特别是来自非日本地区的病例报告和系列，妨碍了诊断的一致性。因此，我们对55例EB-LBCL病例进行了回顾性多机构研究，并对文献中的另外147例病例进行了全面审查，以确定不同的临床病理特征。在我们的研究中，EB-LBCL主要影响老年人（中位年龄80岁），免疫功能正常的患者，表现为淋巴瘤性积液没有固体成分。淋巴瘤性积液多发生于胸膜腔（40/55，73%），其次为心包腔（17/55，31%）。EB-LBCL表达CD 20（53/54，98%）和PAX 5（23/23，100%）。根据Hans算法，大多数病例（30/36，83%）为非生殖中心B细胞亚型。无HHV 8感染（0/55，0%），而在6%（3/47）中检测到EB病毒。临床上，一些患者仅接受引流（15/34，44%），而另一些患者仅接受利妥昔单抗（4/34，12%）或化疗（15/34，44%）。最终，56%（22/39）死亡，中位总生存期（OS）为14.9个月。我们的研究结果与文献中的结果相似;然而，与非日本病例相比，日本病例的心包受累发生率显著较高，化疗率较高，中位OS较长。特别是，我们发现日本居民，心包积液的存在和MYC重排的缺乏都是有利的预后因素。我们的数据表明，EB-LBCL预示着一个更坏的预后比以前报道的，虽然选择患者可以保守治疗。总的来说，EB-LBCL具有独特的临床病理特征，需要建立单独的诊断标准和共识命名法。
Rare cases of human herpesvirus 8 (HHV8)-negative effusion-based large B-cell lymphoma (EB-LBCL) occur in body cavities without antecedent or concurrent solid mass formation. In contrast to HHV8+ primary effusion lymphoma (PEL), EB-LBCL has no known association with HIV or HHV8 infection. However, the small sample sizes of case reports and series worldwide, especially from non-Japanese regions, have precluded diagnostic uniformity. Therefore, we conducted a retrospective, multi-institutional study of 55 cases of EB-LBCL and performed a comprehensive review of an additional 147 cases from the literature to identify distinct clinicopathologic characteristics. In our study, EB-LBCL primarily affected elderly (median age 80 years), immunocompetent patients and manifested as lymphomatous effusion without a solid component. The lymphomatous effusions mostly occurred in the pleural cavity (40/55, 73%), followed by the pericardial cavity (17/55, 31%). EB-LBCL expressed CD20 (53/54, 98%) and PAX5 (23/23, 100%). Most cases (30/36, 83%) were of non-germinal center B-cell subtype per the Hans algorithm. HHV8 infection was absent (0/55, 0%), while Epstein-Barr virus was detected in 6% (3/47). Clinically, some patients were managed with drainage alone (15/34, 44%), while others received rituximab alone (4/34, 12%) or chemotherapy (15/34, 44%). Eventually, 56% (22/39) died with a median overall survival (OS) of 14.9 months. Our findings were similar to those from the literature; however, compared to the non-Japanese cases, the Japanese cases had a significantly higher incidence of pericardial involvement, a higher rate of chemotherapy administration, and longer median OS. Particularly, we have found that Japanese residence, presence of pericardial effusion, and absence of MYC rearrangement are all favorable prognostic factors. Our data suggest that EB-LBCL portends a worse prognosis than previously reported, although select patients may be managed conservatively. Overall, EB-LBCL has distinct clinicopathologic characteristics, necessitating the establishment of separate diagnostic criteria and consensus nomenclature.
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