*National Cancer Institute, Bethesda, MD ‡Massachusetts General Hospital, Boston, MA †British Columbia Cancer Agency, Vancouver, BC, Canada.
Am J Surg Pathol. 2014 Mar;38(3):316-24. doi: 10.1097/PAS.0000000000000107.
Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) and classical Hodgkin lymphoma (CHL) are classified separately because of their distinct clinical and pathologic features. Whereas Epstein-Barr virus (EBV) is detected in the neoplastic cells of 25% to 70% of CHL, NLPHL is generally considered to be EBV(-). We assessed EBV status in 302 pediatric and adult cases of NLPHL. A total of 145 pediatric (age 18 y or younger) and 157 adult cases of NLPHL were retrieved from 3 North American centers and tested for EBV by in situ hybridization (EBV-encoded small RNA). Clinical and pathologic features were analyzed. Five (3.4%) pediatric and 7 (4.5%) adult NLPHL cases contained EBV(+) lymphocyte-predominant (LP) cells. Although all 12 cases met the criteria for diagnosis of NLPHL, atypical features were present, including capsular fibrosis, atrophic germinal centers, and pleomorphic or atypical LP cells. CD20 and OCT-2 were strongly and diffusely positive in all except 1 case. However, PAX5 and CD79a were weak and/or variable in 7/8 and 6/6 cases tested, respectively. EBV(+) cases were more likely to be CD30(+) (75%) compared with EBV(-) cases (25%) (P=0.0007); CD15 was negative in all cases. Our results show that EBV(+) LP cells may occur in NLPHL. Distinguishing EBV(+) NLPHL from CHL can be challenging, as EBV(+) NLPHL can have partial expression of CD30 and weak PAX5 staining as well as pleomorphic-appearing LP cells. However, the overall appearance and maintenance of B-cell phenotype, with strong and diffuse CD20 and OCT-2 expression, support the diagnosis of NLPHL in these cases.
结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）和经典型霍奇金淋巴瘤（CHL）因其独特的临床和病理特征而被分开分类。虽然 25%至 70%的 CHL 肿瘤细胞中可检测到 EBV（Epstein-Barr virus），但 NLPHL 通常被认为是 EBV(-)。我们评估了 302 例儿科和成人 NLPHL 病例的 EBV 状态。从 3 个北美中心共获得 145 例儿科（18 岁或以下）和 157 例成人 NLPHL 病例，并通过原位杂交（EBV 编码的小 RNA）检测 EBV。分析了临床和病理特征。5 例（3.4%）儿科和 7 例（4.5%）成人 NLPHL 病例含有 EBV(+)淋巴细胞为主型（LP）细胞。尽管所有 12 例均符合 NLPHL 的诊断标准，但存在非典型特征，包括包膜纤维化、萎缩性生发中心和多形性或非典型 LP 细胞。除 1 例外，所有病例的 CD20 和 OCT-2 均呈强而弥漫性阳性。然而，在 6/6 例检测的 CD79a 中，7/8 例的 CD79a 呈弱阳性和/或可变。与 EBV(-)病例（25%）相比， EBV(+)病例更可能为 CD30(+)（75%）（P=0.0007）；所有病例 CD15 均为阴性。我们的结果表明，NLPHL 中可能存在 EBV(+) LP 细胞。由于 EBV(+) NLPHL 可能部分表达 CD30，且 CD79a 染色弱阳性和 LP 细胞多形性，因此与 CHL 区分 EBV(+) NLPHL 可能具有挑战性。然而，这些病例中 B 细胞表型的总体表现和维持，具有强而弥漫的 CD20 和 OCT-2 表达，支持 NLPHL 的诊断。
