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鼻腔型结外 NK/T 细胞淋巴瘤,表现为广泛腿部溃疡。

Nasal-type extranodal natural killer/T-cell lymphoma presenting with extensive leg ulcers.

机构信息

Department of Dermatology, National Skin Centre, Singapore.

出版信息

Clin Exp Dermatol. 2009 Dec;34(8):e693-5. doi: 10.1111/j.1365-2230.2009.03428.x. Epub 2009 Jul 3.

DOI:10.1111/j.1365-2230.2009.03428.x
PMID:20055838
Abstract

Primary cutaneous T-cell lymphomas are rare and can be difficult to classify precisely. We present a case of extranodal natural killer (NK)/T-cell lymphoma in a previously healthy, immunocompetent man who presented with extensive necrotic leg ulcers and disseminated skin nodules. Immunohistochemical studies revealed that the tumour cells were positive for CD3, CD30, granzyme B and T-cell intracellular antigen-1, and negative for CD5 and CD56, with positive staining for Epstein-Barr virus (EBV) RNA on in situ hybridization. A diagnosis of extranodal NK/T-cell lymphoma was made, based on the presence of cytotoxic granules and positive EBV RNA staining. The patient was treated with a regimen of chemotherapy comprising corticosteroids, intravenous methotrexate, ifosphamide, L-asparaginase and etoposide with initial response.

摘要

原发性皮肤 T 细胞淋巴瘤较为罕见,且精确分类较为困难。我们报告了一例先前健康、免疫功能正常的男性发生的结外自然杀伤(NK)/T 细胞淋巴瘤,其表现为广泛坏死性腿部溃疡和播散性皮肤结节。免疫组化研究显示,肿瘤细胞 CD3、CD30、颗粒酶 B 和 T 细胞内抗原-1 阳性,CD5 和 CD56 阴性,原位杂交显示 EBV RNA 阳性。基于存在细胞毒性颗粒和 EBV RNA 染色阳性,诊断为结外 NK/T 细胞淋巴瘤。该患者接受了包含皮质类固醇、静脉注射甲氨蝶呤、异环磷酰胺、左旋门冬酰胺酶和依托泊苷的化疗方案治疗,初始反应良好。

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