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诊断模拟白塞病的非鼻型自然杀伤/T 细胞淋巴瘤的挑战:一例病例报告

英文原题:Challenges in Diagnosing Non-Nasal Natural Killer/T-Cell Lymphoma Mimicking Behçet Disease: A Case Report.

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Challenges in Diagnosing Non-Nasal Natural Killer/T-Cell Lymphoma Mimicking Behçet Disease: A Case Report.

PubMed 2025/10/20(内容时间) Am J Case Rep Q3 · IF 0.8(JCR 2025)

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中文摘要

背景 自然杀伤/T细胞淋巴瘤(NKTL)是非霍奇金淋巴瘤的一种独特亚型,与Epstein-Barr病毒感染相关。NKTL可分为鼻型和非鼻型,两者在临床、病理生理和遗传特征上存在显著差异。非鼻型NKTL与Behçet病具有重叠的临床表现,包括复发性溃疡、不明原因发热、腹痛及其他全身症状。在临床实践中区分这两种疾病是一项重大挑战。本报告描述了一例非鼻型NKTL患者,最初被误诊为Behçet病,导致错过了最佳治疗干预。病例报告 一名45岁男性患者,反复出现口腔溃疡伴吞咽痛、吞咽困难和生殖器溃疡1个月。首次住院期间,患者最初被诊断为Behçet病,经皮质类固醇治疗后症状缓解。

然而,尽管规律接受糖皮质激素治疗,3个月内症状复发,疾病进展表现为更大的口腔溃疡、反复发热和消化道出血。进一步的精细血清学、影像学和骨髓穿刺细胞学检查未能获得明确诊断。最终通过同时进行的口腔和回盲部溃疡活检的组织病理学检查确立了NKTL的诊断。

然而,患者拒绝进一步抗肿瘤治疗,并违背医嘱出院。结论 当复发性黏膜皮肤病变与提示淋巴瘤的系统性症状同时出现时,必须尽早进行黏膜和骨髓活检,以确保准确诊断并避免处理不当。提高临床警惕性和全面的病理评估对于区分相似疾病并及时开始治疗至关重要。

展开英文摘要原文

BACKGROUND Natural killer/T-cell lymphoma (NKTL) represents a distinct subtype of non-Hodgkin lymphoma associated with Epstein-Barr virus infection. NKTLs can be classified into nasal or non-nasal forms, which differ substantially in clinical, pathophysiological, and genetic features. Non-nasal-type NKTL and Behçet disease share overlapping clinical manifestations, including recurrent ulcerations, unexplained fever, abdominal pain, and other systemic symptoms. Distinguishing between these 2 diseases poses a significant challenge in clinical practice.

This report describes a patient with non-nasal-type NKTL that was initially misdiagnosed as Behçet's disease, resulting in missed optimal therapeutic intervention. CASE REPORT A 45-year-old man had been suffering from recurrent oral ulcerations accompanied by odynophagia, dysphagia, and genital ulcerations for 1 month. During the first hospitalization, the patient was initially diagnosed with Behçet disease and achieved symptomatic remission following corticosteroid therapy.

However, despite regular glucocorticoid therapy, within 3 months, symptoms recurred, with disease progression manifesting as larger oral ulcers, recurrent fever, and gastrointestinal hemorrhage.

Further refined serological, imaging, and bone marrow aspiration cytology examinations did not yield a definitive diagnosis. The diagnosis of NKTL was finally established through histopathological examination of concurrent oral and ileocecal ulcer biopsies.

However, the patient declined further antitumor therapy and was discharged against medical advice. CONCLUSIONS When recurrent mucocutaneous lesions co-occur with systemic symptoms suggestive of lymphoma, it is critical to pursue early mucosal and bone marrow biopsies to ensure accurate diagnosis and avoid mismanagement. Enhanced clinical vigilance and thorough pathological assessment are essential to distinguish mimicking conditions and initiate timely treatment.

论文信息

作者
Yang X、Yang F、Lu T、Wang J
单位
Department of Nephropathy, Third Xiangya Hospital of Central South University, Changsha, Hunan, China.China
文献类型
病例报告
期刊
The American journal of case reports2025 Oct 20
原文标识
PubMed 41115116 · DOI 10.12659/AJCR.949420