文章：

原发性皮肤间变性大细胞淋巴瘤——临床、形态学、免疫组织化学及分子特征综述

Primary Cutaneous Anaplastic Large Cell Lymphoma—A Review of Clinical, Morphological, Immunohistochemical, and Molecular Features

原文发布日期：14 August 2023

DOI: 10.3390/cancers15164098

类型: Article

开放获取: 是

英文摘要：

Primary cutaneous anaplastic large cell lymphoma (ALCL) is the second most common cutaneous T-cell lymphoma after mycosis fungoides and belongs to the spectrum of cutaneous CD30+ T-cell lymphoproliferative disorders. Although primary cutaneous ALCL usually presents as a localized nodule or papule with or without ulceration, multifocal lesions may occur in up to 20% of cases. Histologically, primary cutaneous ALCL consists of a diffuse dermal infiltrate of medium to large anaplastic/pleomorphic cells with abundant amphophilic-to-eosinophilic cytoplasm, horseshoe-shaped nuclei, strong and diffuse expression of CD30, and with focal or no epidermotropism. The neoplastic infiltrate may show angiocentric distribution and may extend to the subcutis. Patients with localized or multifocal disease have a similar prognosis with a 10-year overall survival rate of 90%. Approximately 30% of primary cutaneous ALCLs harbor aDUSP22(6p25.3) gene rearrangement that results in decreased expression of this dual-specific phosphatase, decreased STAT3 activation, and decreased activity of immune and autoimmune-mediated mechanisms regulated by T-cells.

摘要翻译： 

原发性皮肤间变性大细胞淋巴瘤（ALCL）是仅次于蕈样肉芽肿的第二常见皮肤T细胞淋巴瘤，属于皮肤CD30阳性T细胞淋巴组织增生性疾病谱系。尽管原发性皮肤ALCL通常表现为局限性结节或丘疹，伴或不伴溃疡，但多达20%的病例可出现多灶性病变。组织学上，原发性皮肤ALCL由弥漫性真皮层浸润的中等至大细胞构成，这些细胞呈间变性/多形性，胞质丰富、嗜双色至嗜酸性，核呈马蹄形，CD30呈强且弥漫性表达，表皮趋向性呈局灶性或缺失。肿瘤性浸润可呈血管中心性分布，并可延伸至皮下组织。局限性或多灶性病变患者的预后相似，10年总生存率为90%。约30%的原发性皮肤ALCL存在DUSP22（6p25.3）基因重排，导致该双特异性磷酸酶表达降低、STAT3活化减少，以及T细胞调控的免疫和自身免疫介导机制活性下降。

原文链接：

Primary Cutaneous Anaplastic Large Cell Lymphoma—A Review of Clinical, Morphological, Immunohistochemical, and Molecular Features