CELL INTELLIGENCE · 肿瘤细胞治疗研究
肿瘤细胞治疗研究
英文原题：Dismal Outcome of EBV-Positive Nodal T/NK-Cell Lymphoma: A Multicenter Retrospective Study.
Dismal Outcome of EBV-Positive Nodal T/NK-Cell Lymphoma: A Multicenter Retrospective Study.
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EB-nTNKL 是一种独特的临床病理实体，具有暴发性的临床病程和深度化疗耐药，导致极差的预后。然而，与其他 EBV 相关 T/NK 细胞肿瘤的鉴别，尤其是发生于年轻患者的那些，仍然是诊断上的挑战，需要进一步的临床病理研究。这些不良结局凸显了开发更有效治疗策略的迫切需求。
EBV 阳性结内 T 和 NK 细胞淋巴瘤（EB-nTNKL）是一种新定义的、罕见的、侵袭性 EBV 驱动的淋巴系统恶性肿瘤，通常影响东亚裔老年个体，以男性为主。其特征为细胞毒性 T 细胞表型，迄今记录的病例数量有限。
我们进行了一项多中心回顾性分析，纳入日本11例经淋巴结活检诊断为EB-nTNK L的患者，以更好地描述其临床病理特征和结局。
诊断时的中位年龄为65岁（范围24-81），大多数患者表现为晚期（III/IV）疾病、B症状和高危国际预后指数评分。所有病例均观察到血清乳酸脱氢酶（中位614 U/L）和可溶性白细胞介素-2受体（中位5247 U/mL）水平升高。所有病例均表达细胞毒性分子（颗粒酶B和/或TIA-1），提示细胞毒性T/NK细胞表型，其中8例为CD8阳性。对常规化疗的反应较差。中位总生存期仅为2.9个月，11例患者中有6例在诊断后3个月内死亡。
Epstein-Barr virus (EBV)-positive nodal T- and natural killer (NK)-cell lymphoma (EB-nTNKL) is a newly defined, rare, and aggressive EBV-driven lymphoid malignancy that typically affects older individuals, predominantly male, of East Asian descent. It is characterized by a cytotoxic T-cell phenotype, with only a limited number of cases documented to date.
We performed a multicenter retrospective analysis of 11 patients diagnosed with EB-nTNKL via lymph node biopsy in Japan in order to better characterize its clinicopathological features and outcomes.
The median age at diagnosis was 65 years (range 24-81), and most patients presented with advanced-stage (III/IV) disease, B symptoms, and high-risk International Prognostic Index scores. Elevated serum lactate dehydrogenase (median 614 U/L) and soluble interleukin-2 receptor (median 5247 U/mL) levels were observed in all cases. All cases expressed cytotoxic molecules (granzyme B and/or TIA-1), indicating a cytotoxic T/NK-cell phenotype, and 8 cases were CD8-positive. Responses to conventional chemotherapy were poor. The median overall survival was only 2.9 months, with 6 of 11 patients dying within 3 months of diagnosis.
EB-nTNKL represents a distinct clinicopathological entity with a fulminant clinical course and profound chemoresistance, resulting in an extremely poor prognosis. However, differentiation from other EBV-associated T/NK-cell neoplasms, particularly those arising in younger patients, remains a diagnostic challenge and warrants further clinicopathological investigation. These dismal outcomes underscore an urgent need to develop more effective therapeutic strategies.
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