模仿白塞病的结外NK/T细胞淋巴瘤病例报告。
Case report of extranodal NK/T-cell lymphoma mimicking Behcet syndrome.
作者信息
Park Songyi, Yim Jeemin, Park Hye Eun, Park Jin Hyun, Yoon Hyun-Sun, Kim Jin Soo, Choi In Sil, Kim Young A, Kim Ji Eun, Roh Eun Youn, Kim Ki Hwan
机构信息
Department of Internal Medicine, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Republic of Korea.
Department of Pathology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Republic of Korea.
出版信息
Medicine (Baltimore). 2025 Sep 5;104(36):e44329. doi: 10.1097/MD.0000000000044329.
RATIONALE
Extranodal natural killer (NK)/T-cell lymphoma is an uncommon non-Hodgkin lymphoma, prevalent in Asia. It often involves the nasal and upper airway regions but can disseminate to other sites like skin, soft tissue, testis, and gastrointestinal tract, characterized by Epstein-Barr virus association.
PATIENT CONCERNS
This report discusses a 48-year-old male initially diagnosed with Behcet syndrome with dry mouth, uveitis, pruritic macules, and human leukocyte antigen-B51 positivity. Despite steroid treatment, a growing testicular mass and erythema nodosum led to reevaluation.
DIAGNOSES
Biopsy confirmed Epstein-Barr virus-positive extranodal NK/T-cell lymphoma. The imaging indicated lymphoma spread to the nasal cavity, lymphatic chains, bones, and spleen.
INTERVENTIONS
The patient received multi-agent SMILE (methotrexate, ifosfamide, L-asparaginase, mesna, etoposide, dexamethasone) chemotherapy.
OUTCOMES
The patient achieved a metabolic complete response after 2 cycles of SMILE chemotherapy.
LESSONS
This report highlights the diagnostic pitfalls due to symptoms and signs overlapping between Behcet syndrome and extranodal NK/T-cell lymphoma.
理论依据
结外自然杀伤（NK）/T细胞淋巴瘤是一种罕见的非霍奇金淋巴瘤，在亚洲较为常见。它常累及鼻腔和上呼吸道区域，但也可播散至皮肤、软组织、睾丸和胃肠道等其他部位，其特征为与EB病毒相关。
患者情况
本报告讨论了一名48岁男性，最初被诊断为白塞病，伴有口干、葡萄膜炎、瘙痒性黄斑和人类白细胞抗原B51阳性。尽管接受了类固醇治疗，但不断增大的睾丸肿块和结节性红斑导致了重新评估。
诊断
活检证实为EB病毒阳性的结外NK/T细胞淋巴瘤。影像学检查显示淋巴瘤已扩散至鼻腔、淋巴链、骨骼和脾脏。
干预措施
患者接受了多药联合的SMILE（甲氨蝶呤、异环磷酰胺、L-天冬酰胺酶、美司钠、依托泊苷、地塞米松）化疗。
结果
患者在接受2个周期的SMILE化疗后达到代谢完全缓解。
经验教训
本报告强调了由于白塞病和结外NK/T细胞淋巴瘤的症状和体征重叠而导致的诊断陷阱。
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