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鼻型结外NK/T细胞淋巴瘤,表现为酷似肉芽肿性脂膜炎的原发性皮肤损害:1例病例报告并文献复习

Extranodal NK/T cell lymphoma, nasal type, presenting with primary cutaneous lesion mimicking granulomatous panniculitis: a case report and review of literature.

作者信息

Sitthinamsuwan Panitta, Pongpruttipan Tawatchai, Chularojmontri Leena, Pattanaprichakul Penvadee, Khuhapinant Archrob, Sukpanichnant Sanya

机构信息

Department of Pathology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

出版信息

J Med Assoc Thai. 2010 Aug;93(8):1001-7.

Abstract

BACKGROUND

Cutaneous extranodal NK/T-cell lymphoma, nasal type (NK/T) is relatively rare, associated with aggressive behavior and poor prognosis. Histopathological findings, immunohistochemical study and EBV-encoded RNA (EBER) in situ hybridization are essential for the diagnosis.

CASE REPORT

A 54-year-old Thai man with NK/T of the nasal cavity initially presented with cutaneous NK/T mimicking granulomatous panniculitis. The skin biopsies were performed twice due to the marked necrosis in the first one. The second biopsy revealed small, medium, and large atypical lymphoid cells infiltrating fat lobules with necrotic foci and granulomatous reaction. Within the granulomatous inflammation, the atypical lymphoid cells showing involvement of the blood vessel (angiocentricity) were noted. Immunostaining demonstrated that the atypical lymphoid cells marked with CD3, CD56, and TIA-1, but they did not mark with CD5, CD20, CD15, or CD30. EBER in situ hybridization was positive.

CONCLUSION

Cutaneous NK/T can produce granulomatous panniculitis. The recognition of atypical lymphoid cells showing angiocentricity together with immunohistochemistry and EBER in situ hybridization are crucial for the correct diagnosis.

摘要

背景

皮肤结外NK/T细胞淋巴瘤,鼻型(NK/T)相对罕见,具有侵袭性生物学行为且预后较差。组织病理学检查结果、免疫组化研究及EB病毒编码RNA(EBER)原位杂交对诊断至关重要。

病例报告

一名54岁泰国男性,鼻腔NK/T淋巴瘤初诊时表现为皮肤NK/T,酷似肉芽肿性脂膜炎。因首次皮肤活检有明显坏死,故进行了两次活检。第二次活检显示有小、中、大非典型淋巴细胞浸润脂肪小叶,伴有坏死灶及肉芽肿反应。在肉芽肿性炎症中,可见累及血管(血管中心性)的非典型淋巴细胞。免疫组化显示非典型淋巴细胞表达CD3、CD56和TIA-1,但不表达CD5、CD20、CD15或CD30。EBER原位杂交呈阳性。

结论

皮肤NK/T可表现为肉芽肿性脂膜炎。识别具有血管中心性的非典型淋巴细胞以及免疫组化和EBER原位杂交对正确诊断至关重要。

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