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伴有显著坏死的痤疮样原发性皮肤CD4阳性小/中等多形性T细胞淋巴瘤

Acneiform primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoma with prominent necrosis.

作者信息

Wang Lei, Wang Gang, Gao Tianwen

机构信息

Department of Dermatology, Xijing Hospital, Fourth Military Medical University, Xian, China.

出版信息

J Cutan Pathol. 2015 Apr;42(4):265-70. doi: 10.1111/cup.12443. Epub 2015 Feb 17.

DOI:10.1111/cup.12443
PMID:25407448
Abstract

Primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoma (SMPTCL) is an indolent form of cutaneous lymphoma that usually presents in solitary fashion and is histopathologically characterized by nodular infiltration of small- to medium-sized pleomorphic T-cells. We report the case of a patient who presented with a 5-year history of acneiform lesions on his face. Histopathologic examination of two lesions revealed a nodular infiltrate of small to medium-sized lymphocytes with necrosis in the dermis. The proliferating cells were positive for CD2, CD3 and CD4 and negative for CD8, CD30 and CD56. They were positive for TIA-1 and negative for perforin and granzyme B. The Ki67 proliferation index was approximately 10%. The neoplastic cells expressed programmed death-1 and lacked expression of CXCL-13, bcl-6 and CD10. In situ hybridization for Epstein-Barr virus-encoded RNA yielded a negative result. T-cell receptor gene rearrangement showed identical T-lymphocyte monoclonality in both lesions. In brief, we report a rare case of acneiform SMPTCL with prominent necrosis.

摘要

原发性皮肤CD4阳性小/中多形性T细胞淋巴瘤(SMPTCL)是一种惰性皮肤淋巴瘤,通常以单发形式出现,组织病理学特征为小至中等大小多形性T细胞的结节状浸润。我们报告一例患者,其面部有5年的痤疮样皮损病史。对两处皮损进行组织病理学检查,发现真皮内有小至中等大小淋巴细胞的结节状浸润并伴有坏死。增殖细胞CD2、CD3和CD4呈阳性,CD8、CD30和CD56呈阴性。它们TIA-1呈阳性,穿孔素和颗粒酶B呈阴性。Ki67增殖指数约为10%。肿瘤细胞表达程序性死亡-1,缺乏CXCL-13、bcl-6和CD10的表达。爱泼斯坦-巴尔病毒编码RNA的原位杂交结果为阴性。T细胞受体基因重排显示两处皮损中T淋巴细胞单克隆性相同。简而言之,我们报告了一例罕见的伴有明显坏死的痤疮样SMPTCL病例。

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