Abro Brooj, Allen Pamela, Asakrah Saja, Bradley Kyle, Zhang Linsheng
Department of Pathology and Laboratory Medicine, Emory University School of Medicine, 1364 Clifton Road NE, Atlanta, GA 30322, USA.
Department of Hematology and Oncology, Winship Cancer Institute of Emory University, 1365 Clifton Road NE, Atlanta, GA 30322, USA.
Hematol Rep. 2024 May 23;16(2):308-316. doi: 10.3390/hematolrep16020031.
EBV-positive nodal T- and NK-cell lymphoma (EBV+ NT/NKCL) is a recently recognized entity in the 5th edition of the WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues. Notably, CD30 positivity is frequently observed in (EBV+ NT/NKCL), creating diagnostic challenges to distinguish it from ALK-negative anaplastic large cell lymphoma (ALCL). Furthermore, cases of EBV+ ALCL have been documented in the literature, predating the inclusion of EBV+ nodal cytotoxic T-cell lymphoma as a variant of peripheral T-cell lymphoma. We present a case of a 47-year-old male presenting with multiple lymphadenopathies. The histomorphologic and immunophenotypic features of the lymph node closely resemble ALK-negative ALCL, characterized by uniform CD30 expression and a subcapsular distribution of lymphoma cells. However, the lymphoma cells exhibit diffuse positivity for EBV, consistent with EBV+ NT/NKCL. A case of ALK-negative ALCL with an immunophenotype identical to the EBV-positive case is included for comparison. Given that EBV+ NT/NKCL represents an aggressive neoplasm requiring unique clinical management compared to ALK-negative ALCL, it is critical to accurately differentiate EBV+ NT/NKCL from ALK-negative ALCL with a cytotoxic T-cell immunophenotype.
EBV 阳性的淋巴结 T 细胞和 NK 细胞淋巴瘤(EBV+ NT/NKCL)是世界卫生组织造血与淋巴组织肿瘤分类第 5 版中最近才被认识的一种疾病实体。值得注意的是,在(EBV+ NT/NKCL)中经常观察到 CD30 阳性,这给将其与 ALK 阴性间变性大细胞淋巴瘤(ALCL)区分开来带来了诊断挑战。此外,文献中已记录了 EBV+ ALCL 的病例,早于将 EBV+ 淋巴结细胞毒性 T 细胞淋巴瘤作为外周 T 细胞淋巴瘤的一种变体纳入分类之前。我们报告一例 47 岁男性,表现为多处淋巴结肿大。淋巴结的组织形态学和免疫表型特征与 ALK 阴性 ALCL 非常相似,其特征为 CD30 表达均匀且淋巴瘤细胞呈包膜下分布。然而,淋巴瘤细胞对 EBV 呈弥漫性阳性,符合 EBV+ NT/NKCL。为作比较,纳入了一例免疫表型与 EBV 阳性病例相同的 ALK 阴性 ALCL。鉴于与 ALK 阴性 ALCL 相比,EBV+ NT/NKCL 是一种需要独特临床管理的侵袭性肿瘤,准确区分具有细胞毒性 T 细胞免疫表型的 EBV+ NT/NKCL 和 ALK 阴性 ALCL 至关重要。