Alberti-Violetti Silvia, Torres-Cabala Carlos A, Talpur Rakhshandra, Corti Laura, Fanoni Daniele, Venegoni Luigia, Berti Emilio, Duvic Madeleine
UOC Dermatologia, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Milan, Italy.
Department of Pathology, Dermatopathology Section, University of Texas, MD Anderson Cancer Center, Houston, TX, USA.
J Cutan Pathol. 2016 Dec;43(12):1121-1130. doi: 10.1111/cup.12806. Epub 2016 Sep 20.
Primary cutaneous CD4+ small-/medium-sized pleomorphic T-cell lymphoma (CD4+ PCSM-TCL) is a rare lymphoproliferative disorder with a favorable prognosis. Distinguishing it from other cutaneous lymphomas is often a challenge.
We retrospectively collected CD4+PCSM-TCL cases from two centers (MD Anderson Cancer Center, USA and University of Milan, Italy) and evaluated their clinicopathological features. Array-comparative genomic hybridization (aCGH) analysis was performed on 11 cases.
A total of 62 patients were identified. Single lesions were the most common clinical presentations (79%). Five patients (8%) showed multiple MF-like plaques. All patients' disease had an indolent course. The infiltrate was nodular and diffuse, multinodular or superficial but in all cases, it was characterized by small/medium pleomorphic CD4+/CD279(PD1+) lymphocytes grouped in clusters and 'pseudorosettes' around B-cells. aCGH analysis showed no significant genomic abnormalities. Single lesions were mainly treated with surgical excision (91%) and/or radiotherapy (95%) with low rate of relapse (12%). For multiple lesions, topical steroids, nitrogen mustard and phototherapy were mainly used but the rate of relapse was high (69%).
CD4+PCSM-TCL is characterized by heterogeneous clinical presentations. The arrangement of atypical cells in clusters or pseudorosettes is a useful criterion for diagnosis. The absence of significant genomic alterations is in agreement with its indolent behavior.
原发性皮肤CD4+小/中等大小多形性T细胞淋巴瘤（CD4+ PCSM-TCL）是一种罕见的淋巴增殖性疾病，预后良好。将其与其他皮肤淋巴瘤区分开来往往具有挑战性。
我们回顾性收集了来自两个中心（美国MD安德森癌症中心和意大利米兰大学）的CD4+PCSM-TCL病例，并评估了其临床病理特征。对11例病例进行了阵列比较基因组杂交（aCGH）分析。
共鉴定出62例患者。单个皮损是最常见的临床表现（79%）。5例患者（8%）表现为多个蕈样肉芽肿样斑块。所有患者的疾病病程呈惰性。浸润呈结节状和弥漫性、多结节状或浅表性，但在所有病例中，其特征是小/中等多形性CD4+/CD279（PD1+）淋巴细胞聚集成簇，并围绕B细胞形成“假菊形团”。aCGH分析显示无明显基因组异常。单个皮损主要采用手术切除（91%）和/或放疗（95%），复发率较低（12%）。对于多个皮损，主要使用外用类固醇、氮芥和光疗，但复发率较高（69%）。
CD4+PCSM-TCL具有异质性临床表现。非典型细胞成簇或形成假菊形团的排列是诊断的有用标准。无明显基因组改变与其惰性行为一致。
