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鼻型结外 NK/T 细胞淋巴瘤表现为非典型皮肤缺损:一例病例报告

英文原题:Nasal type of extranodal NK/T-cell lymphoma presenting as an atypical skin defect: A case report.

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Nasal type of extranodal NK/T-cell lymphoma presenting as an atypical skin defect: A case report.

PubMed 2026/07/03(内容时间) Medicine (Baltimore) Q2 · IF 2(JCR 2025)

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研究思路按摘要原文分段

鼻型结外NK/T细胞淋巴瘤(ENKTL)是一种主要累及上呼吸消化道的罕见恶性肿瘤。它与EB病毒(EBV)感染密切相关;因此,EBV阴性病例极为罕见,其临床病理特征和治疗反应尚未明确。患者主诉:一名75岁男性因左眼内眦区持续性皮肤缺损就诊。他因疑似蜂窝织炎接受抗生素治疗2个月,但症状持续,皮肤缺损加重。诊断:手术清创发现一个形状不规则的白色的肿块延伸至鼻旁窦。切开活检显示非典型淋巴细胞浸润,符合ENKTL。EBV原位杂交阴性,支持EBV阴性ENKTL的诊断。分期检查,包括全身计算机断层扫描和正电子发射断层扫描,显示病变局限于眼眶和鼻旁窦。干预:患者接受依托泊苷、异环磷酰胺、顺铂和地塞米松化疗。第一个周期后,患者出现严重中性粒细胞减少,第二个周期化疗剂量减少40%。在2个周期的依托泊苷、异环磷酰胺、顺铂和地塞米松化疗后,建议进行巩固放疗。结果:2个周期化疗后,正电子发射断层扫描显示代谢完全缓解。第二个周期化疗后3个月,皮肤缺损完全愈合,随访计算机断层扫描显示疾病缓解。经验教训:ENKTL的皮肤表现可模拟蜂窝织炎等良性炎症性疾病,导致诊断延迟。临床医生应保持高度警惕,对眶周和鼻部的不典型溃疡性病变尽早进行组织活检,即使缺乏典型的EBV阳性。

展开英文摘要原文

RATIONALE: Nasal-type extranodal natural killer/T-cell lymphoma (ENKTL) is a rare malignancy primarily affecting the upper aerodigestive tract. It is strongly associated with Epstein-Barr virus (EBV) infection; thus, EBV-negative cases are extremely rare, and their clinicopathological features and treatment responses are not well defined. PATIENT CONCERNS: A 75-year-old male presented with a persistent skin defect in the medial canthal area of the left eye. He had been treated with antibiotics for presumed cellulitis for 2 months, but the symptoms persisted, and the skin defect worsened. DIAGNOSES: Surgical debridement revealed an irregularly shaped white mass extending into the paranasal sinus. Incisional biopsy revealed atypical lymphocytic infiltration favoring ENKTL. EBV in situ hybridization was negative, supporting the diagnosis of EBV-negative ENKTL. Staging examinations, including whole-body computed tomography and positron emission tomography, revealed localized disease involving only the orbit and paranasal sinuses.

INTERVENTIONS: The patient was treated with etoposide, ifosfamide, cisplatin, and dexamethasone chemotherapy. After the first cycle, the patient developed severe neutropenia, and the chemotherapy dose was reduced by 40% for the second cycle. After 2 cycles of etoposide, ifosfamide, cisplatin, and dexamethasone chemotherapy, consolidation radiation therapy was recommended. OUTCOMES: After 2 cycles of chemotherapy, positron emission tomography showed metabolic complete remission.

At 3 months after the second cycle of chemotherapy, the skin defect had completely healed, and follow-up computed tomography showed disease remission. LESSONS: Cutaneous manifestations of ENKTL can mimic benign inflammatory conditions such as cellulitis, leading to delayed diagnosis. Clinicians should maintain a high index of suspicion and perform early tissue biopsy for atypical ulcerative lesions in the periorbital and nasal areas, even in the absence of typical EBV positivity.

论文信息

作者
Kim YJ、Park IK、Nam SH、Won KY、Shin JH、Moon SW、Chung J
单位
Department of Ophthalmology, Kyung Hee University Hospital at Gangdong, Kyung Hee University, College of Medicine, Seoul, Korea.South Korea
文献类型
病例报告
期刊
Medicine2026 Jul 3
原文标识
PubMed 42410802 · DOI 10.1097/MD.0000000000049584