From the Department of Pathology (Menke, Natkunam, Warnke, Gratzinger), at Stanford University Medical Center, Stanford, California.
Division of Oncology, Department of Medicine (Spinner, Advani), at Stanford University Medical Center, Stanford, California.
Arch Pathol Lab Med. 2021 Jun 1;145(6):753-758. doi: 10.5858/arpa.2020-0135-OA.
CONTEXT.—: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare, indolent Hodgkin lymphoma subtype with distinct clinicopathologic features and treatment paradigms. The neoplastic lymphocyte-predominant cells typically express bright CD20 and other B-cell antigens, which distinguishes them from Hodgkin/Reed-Sternberg cells of lymphocyte-rich classic Hodgkin lymphoma.
OBJECTIVE.—: To characterize the clinicopathologic features of CD20-negative NLPHL at a single institution.
DESIGN.—: A retrospective search for CD20-negative NLPHL in our pathology archives and medical records was conducted.
RESULTS.—: Of 486 NLPHL patients identified with CD20 available for review, 14 (2.8%) had LP cells with absent CD20 expression. Patients with prior rituximab administration (n = 7) and insufficient clinical history (n = 1) were excluded, leaving 6 patients with rituximab-naïve, CD20-negative NLPHL. A broad immunohistochemical panel showed the LP cells in all cases expressed B-cell antigens, particularly Oct-2, although PAX5 and CD79a were frequently also dim. CD30, CD15, and Epstein-Barr virus-encoded small RNAs were negative in all evaluated cases. Two patients had high-risk variant immunoarchitectural pattern D. One patient had extranodal disease, involving the spleen and bone, and was suspected to have large cell transformation. Standard NLPHL therapy was given, including local radiation and/or chemotherapy. Of 5 patients with available follow-up, 4 are alive in complete remission after therapy, and 1 is alive with relapsed disease.
CONCLUSIONS.—: NLPHL can lack CD20 de novo without prior rituximab therapy. In such cases, extensive immunophenotyping helps distinguish NLPHL from lymphocyte-rich classic Hodgkin lymphoma, which differ in clinical behavior and therapy. In our series, CD20-negative NLPHL showed both classic and variant histologic patterns and the expected range of clinical behavior seen in NLPHL, including 1 case with suspected large cell transformation.
结节性淋巴细胞为主型霍奇金淋巴瘤（NLPHL）是一种罕见的、惰性的霍奇金淋巴瘤亚型，具有独特的临床病理特征和治疗模式。肿瘤性淋巴细胞为主的细胞通常表达明亮的 CD20 和其他 B 细胞抗原，这将其与富含淋巴细胞的经典霍奇金淋巴瘤中的霍奇金/里斯滕伯格细胞区分开来。
描述单个机构中 CD20 阴性 NLPHL 的临床病理特征。
对我们的病理档案和病历中进行了 CD20 阴性 NLPHL 的回顾性搜索。
在 486 例可用于回顾的 CD20 阳性 NLPHL 患者中，有 14 例（2.8%）的 LP 细胞 CD20 表达缺失。排除了先前接受利妥昔单抗治疗的患者（n=7）和临床病史不足的患者（n=1），共 6 例初治 CD20 阴性 NLPHL 患者纳入研究。广泛的免疫组化检测显示，所有病例的 LP 细胞均表达 B 细胞抗原，特别是 Oct-2，尽管 PAX5 和 CD79a 也常为弱表达。所有评估病例的 CD30、CD15 和 EBV 编码的小 RNA 均为阴性。2 例患者具有高危变异免疫组织学模式 D。1 例患者有结外疾病，累及脾脏和骨骼，怀疑发生大细胞转化。给予标准 NLPHL 治疗，包括局部放疗和/或化疗。在可获得随访的 5 例患者中，4 例在治疗后完全缓解，1 例疾病复发后仍存活。
NLPHL 可在没有先前利妥昔单抗治疗的情况下初次出现 CD20 缺失。在这种情况下，广泛的免疫表型有助于将 NLPHL 与淋巴细胞丰富的经典霍奇金淋巴瘤区分开来，后者在临床行为和治疗上存在差异。在我们的研究中，CD20 阴性 NLPHL 显示出经典和变异的组织学模式，以及在 NLPHL 中预期的一系列临床行为，包括 1 例疑似大细胞转化。
