原发皮肤CD30阳性间变大细胞淋巴瘤七例临床病理分析

Clinical and pathological analysis of 7 cases of primary cutaneous CD30＋ anaplastic large cell lymphoma

目的探讨原发皮肤CD30阳性间变大细胞淋巴瘤（PC—ALCL）的临床与组织病理学特征。方法回顾性分析7例PC—ALCL患者的临床及病理资料情况。结果7例患者中，男6例，女1例，平均发病年龄52岁。皮损为红色结节、肿块和（或）斑块，3例多发，4例单发，6例伴溃疡形成。所有患者均未见系统受累。组织病理改变：肿瘤细胞在真皮内弥漫性分布，细胞体积大，胞质丰富，胞核呈异形改变，可见核分裂象。肿瘤细胞CD30和细胞毒蛋白阳性，CD20、CD56、间变性淋巴瘤激酶（ALK）和EB病毒编码小RNA原位杂交均阴性。结论PC—ALCL是一种少见的原发于皮肤的低度恶性T细胞淋巴瘤，结合临床表现、皮损组织病理及免疫组化检查可确诊，罕见系统受累及转移。

Objective To investigate clinical and pathological features ofprimary cutaneous CD30＋ anaplastic large cell lymphoma （PC-ALCL）. Methods Clinical and pathological data were collected from 7 patients with PC-ALCL and analyzed retrospectively. Results Of the 7 patients, 6 were male and 1 was female, with an average age of 52 years. PC-ALCL was characterized by solitary （n = 3 ） or multiple （n = 4） erythematous nodules, lumps and/or plaques with （n = 6） or without （n = 1 ） ulceration. Systemic involvement was observed in none of the 7 patients. Histopathological examination showed diffuse distribution of tumor cells in the dermis, which were large with rich cytoplasm and atypical nuclei. Mitotic figures were seen. An immunohistoehemieal study of tumor cells showed positive staining for CD30 and cytotoxic protein, but negative staining for CD20, CD56, anaplastic lymphoma kinase （ALK）. Epstein-Barr virus-encoded RNA in situ hybridization was negative. Conclusions PC-ALCL is a rare primary cutaneous low-grade malignant T-cell lymphoma, which can be confirmed by clinical manifestations as well as histopathological and immunohistochemical examinations. It usually has good prognosis with rare systemic involvement and metastasis.