Departments of Pathology and Laboratory Medicine.
Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine.
Am J Surg Pathol. 2020 Sep;44(9):1173-1183. doi: 10.1097/PAS.0000000000001499.
Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoproliferation encompasses a broad range of clinicopathologic findings, including specific subtypes, for example, EBV mucocutaneous ulcer. Here we reassessed 36 cases of primary EBV diffuse large B-cell lymphomas (16 men and 20 women; median age, 69.5 y; range, 35 to 84 y), including 8 immunosuppressed patients (Lugano stage II-IV; median age, 74 y), 7 nonimmunosuppressed patients with stage I disease (median age, 69 y), and 21 nonimmunosuppressed patients with stage II-IV disease (median age, 69 y). All immunosuppressed patients exhibited iatrogenic immunodeficiency and an ulcerative appearance, with ulcer sites including the stomach (1 patient), small intestine (6 patients), and rectum (1 patient). Four patients were in the setting of treated lymphoma-associated immunosuppression. Immunosuppressed patients had higher incidences of intestinal involvement (P=0.001) and perforation (n=2) compared with advanced stage nonimmunosuppressed patients. Among nonimmunosuppressed stage I patients, lesions were restricted to the stomach, none showed multiple lesions or elevated serum lactate dehydrogenase, and the overall survival curve plateaued, although it was not statistically significant (P=0.0581). One nonimmunosuppressed stage I patient with a polypoid lesion exhibited spontaneous regression within 2 months after diagnosis, while another with bulky disease pursued an aggressive clinical course. Nonimmunosuppressed stage I cases without bulky masses may be considered EBV mucocutaneous ulcer with local progression. Our results demonstrated that primary EBV gastrointestinal diffuse large B-cell lymphoma could be delineated into 3 groups based on immune status and clinical stage, revealing distinguishing features useful as a pragmatic guide for diagnostic and therapeutic approaches.
EB 病毒（EBV）阳性弥漫性大 B 细胞淋巴瘤的临床表现范围广泛，包括特定的亚型，例如 EBV 黏膜溃疡性疾病。在这里，我们重新评估了 36 例原发性 EBV 弥漫性大 B 细胞淋巴瘤患者（16 名男性和 20 名女性；中位年龄 69.5 岁；范围 35 至 84 岁），包括 8 例免疫抑制患者（卢加诺分期 II-IV 期；中位年龄 74 岁）、7 例无免疫抑制的 I 期疾病患者（中位年龄 69 岁）和 21 例无免疫抑制的 II-IV 期疾病患者（中位年龄 69 岁）。所有免疫抑制患者均存在医源性免疫缺陷和溃疡性表现，溃疡部位包括胃（1 例）、小肠（6 例）和直肠（1 例）。4 例患者为治疗相关的淋巴瘤相关性免疫抑制。与晚期非免疫抑制患者相比，免疫抑制患者肠道受累（P=0.001）和穿孔（n=2）的发生率更高。在非免疫抑制的 I 期患者中，病变局限于胃，无多发性病变或血清乳酸脱氢酶升高，总生存曲线呈平台期，尽管无统计学意义（P=0.0581）。1 例非免疫抑制的 I 期患者，其息肉样病变在诊断后 2 个月内自发消退，而另 1 例肿块较大的患者病情进展迅速。无肿块的非免疫抑制的 I 期病例可能被认为是局部进展的 EBV 黏膜溃疡性疾病。我们的结果表明，原发性 EBV 胃肠道弥漫性大 B 细胞淋巴瘤可根据免疫状态和临床分期分为 3 组，这些特征有助于为诊断和治疗方法提供实用的指导。
