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结节性淋巴细胞为主型霍奇金淋巴瘤的地理变异。

Geographic Variability of Nodular Lymphocyte-Predominant Hodgkin Lymphoma.

机构信息

Division of Hematopathology and Transfusion Medicine, University Health Network, Toronto, Canada.

Aga Khan University Hospital, Nairobi, Kenya.

出版信息

Am J Clin Pathol. 2022 Feb 3;157(2):231-243. doi: 10.1093/ajcp/aqab113.

Abstract

OBJECTIVES

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) differs from classic Hodgkin lymphoma (CHL) in terms of clinicopathologic features, including Epstein-Barr virus (EBV) association. CHL geographic variability is well known, with higher frequencies of mixed-cellularity subtype and EBV positivity in low/middle-income countries (LMICs), but there are few well-characterized series of NLPHL from LMICs.

METHODS

We detail clinicopathologic findings of 21 NLPHL cases received in consultation from Kenya and summarize reports of NLPHL with EBV testing published since 2000.

RESULTS

Median age of consultation cases was 36 years, and male/female ratio was 3.2. All cases involved peripheral lymph nodes and showed at least some B-cell-rich nodular immunoarchitecture, with prominent extranodular lymphocyte-predominant (LP) cells and T-cell-rich variant patterns most commonly seen. LP cells expressed pan-B-cell markers, including strong OCT2; lacked CD30 and CD15 expression in most cases; and were in a background of expanded/disrupted follicular dendritic cell meshworks and increased T-follicular helper cells. LP cells were EBV negative in 18 cases. Historical cases showed a low rate of EBV positivity with no significant difference between LMICs and high-income countries.

CONCLUSIONS

Unlike CHL, NLPHL shows few geographic differences in terms of clinicopathologic features and EBV association. These findings have implications for diagnosis, prognostication, and treatment of patients with NLPHL in LMICs.

摘要

目的

结节性淋巴细胞为主型霍奇金淋巴瘤(NLPHL)在临床病理特征方面与经典霍奇金淋巴瘤(CHL)不同,包括 EBV 相关性。CHL 的地理变异性是众所周知的,在低收入和中等收入国家(LMICs)中,混合细胞亚型和 EBV 阳性率较高,但来自 LMICs 的 NLPHL 特征良好的系列研究很少。

方法

我们详细描述了从肯尼亚会诊的 21 例 NLPHL 病例的临床病理发现,并总结了自 2000 年以来发表的具有 EBV 检测的 NLPHL 报告。

结果

会诊病例的中位年龄为 36 岁,男女比例为 3.2。所有病例均累及外周淋巴结,至少存在一些 B 细胞丰富的结节性免疫组织学结构,最常见的是明显的结节外淋巴细胞为主(LP)细胞和 T 细胞丰富的变异型。LP 细胞表达泛 B 细胞标志物,包括强烈的 OCT2;在大多数情况下缺乏 CD30 和 CD15 的表达;并存在滤泡树突状细胞网状结构扩张/破坏和 T 滤泡辅助细胞增加的背景。18 例 LP 细胞 EBV 阴性。历史病例显示 EBV 阳性率较低,LMICs 与高收入国家之间无显著差异。

结论

与 CHL 不同,NLPHL 在临床病理特征和 EBV 相关性方面几乎没有地理差异。这些发现对 NLPHL 患者在 LMICs 的诊断、预后和治疗具有重要意义。

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