原发性皮肤CD30阳性淋巴组织增生性疾病临床病理分析

目的 探讨原发性皮肤CD30阳性淋巴组织增生性疾病的临床及病理学特征。方法 对4例淋巴瘤样丘疹病及5例原发性皮肤间变性大细胞淋巴瘤的临床、病理学特征及免疫组化表达进行分析。结果 淋巴瘤样丘疹病分为A、B、C三型，组织学上形成一个连续的谱系，A型见多形性间变性大细胞或Sternberg-Reed样细胞散在分布或小片状分布在多量炎性细胞背景中；B型类似蕈样肉芽肿病变，表现为真皮层较宽的淋巴细胞浸润带，其中散在少量小至中等异形脑回样淋巴细胞；C型以间变性大细胞弥漫分布为特征，但临床上可自行消退。原发性皮肤间变性大细胞以皮下结节或皮肤丘疹就诊，瘤细胞体积大，呈多形性、圆形或椭圆形，胞质丰富，嗜酸或呈双色性，核大，核仁明显。两组病变中的大细胞均特征性表达CD30，预后均较好。结论 原发性皮肤CD30阳性淋巴组织增生性疾病是一组预后较好的皮肤T细胞性淋巴瘤谱系，综合临床表现、组织病理改变、免疫组化有助于本病的诊断。

Primary cutaneous CD30 positive lymphoproliferative disorders: a clinicopathologic analysis

Objective To analyze the clinicopathologic features of primary cutaneous CD30 positive lymphoproliferative disorders. Methods A clinical, pathological and immunohistochemical analysis was carried out in 4 cases of lymphomatoid papulosis and 5 cases of primary cutaneous anaplastic large cell lymphoma. Results Lymphomatoid papulosis was divided into 3 subtypes, A, B and C. The lymphomatoid papulosis of subtype A was pathologically characterized by pleomorphic anaplastic large cells or Steinberg-reed cells scattered or patchly distributed among many inflammatory cells; subtype B showed pathological changes characteristic of granuloma fungoides, and manifested as a broad infiltration zone of lymphocytes in dermis with scattered small- to middle-sized atypical gyrus-like lymphocytes; subtype C was characterized by a diffuse distribution of anaplastic large cells and could clinically subside spontaneously. Primary cutaneous anaplastic large cell lymphoma clinically manifested as subcutaneous nodules or papules, and was pathologically characterized by large, pleomorphic, round or ellipse cells with plentiful, eosinophilic or bicolor cytoplasm, large nuclei and obvious nucleoli. The neoplastic cells characteristically expressed CD30 antigen in both lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, and all the cases showed a favorable prognosis.Conclusions Primary cutaneous CD30 positive lymphoproliferative disorders are a spectrum of cutaneous T cell lymphoma with favorable prognosis, and a synthetic analysis of clinical, histopathological and immunohistochemical findings is beneficial to the diagnosis of these entities.