目的探讨皮肤淋巴组织增生性病变的临床病理特征,以期提高病理诊断质量,规范临床治疗。方法复习16例皮肤淋巴组织增生性病变患者临床病理资料,分析常见皮肤淋巴组织增生性病变的基本构成、大体皮损及组织学形态、免疫表型特点,探讨病理诊断和诊断要点。结果16例患者男性11例,女性5例,男女比2.2:1,中位年龄52.5岁。病程2月~16年不等,临床皮损形态多样且无特异性。病理诊断为非肿瘤性的皮肤淋巴细胞浸润3例,淋巴瘤13例包括蕈样霉菌病4例、淋巴瘤样丘疹病3例、原发皮肤间变性大细胞淋巴瘤2例、ALK阳性间变性大细胞淋巴瘤累及皮肤1例、皮肤NK/T细胞淋巴瘤1例,弥漫大B细胞淋巴瘤2例。10例行EBER1/2原位杂交检测,仅1例皮肤NK/T细胞淋巴瘤阳性。9例行TCR7基因或IgH/IgK受体基因重排分析,蕈样霉菌病1例、间变性大细胞淋巴瘤2例、淋巴瘤样丘疹病1例和弥漫大B细胞淋巴瘤1例检出克隆性重排。结论本组皮肤淋巴组织增生病变中,T细胞淋巴瘤居多,以蕈样霉菌病最为常见;与EB病毒关系不密切;诊断与鉴别诊断需密切结合临床。
Objective To investigate the clinicpathologic features of cutaneous lymphoproliferative disorder. Methods 16 cases of cutaneous lymphoproliferative disorders were collected and analyzed. Constitution, skin eruptions, histopathologic feature, immunophenotype, Epstein-Barr virus status and clinical course were studied. Results Among the 16 patients, the ratio of male to female was 2.2: 1. Median age was 52.5 years old. Clinical courses of disease ranged from 2 years to 16 years. Various and non-specific skin eruptions were observed. 16 case were diagnosed as lymphocytic infilitration of skin (3 cases), mycosis fungiodes(4 cases), primary cutaneous anaplastic large cell lymphoma(2 cases), ALK positive anaplastic large cell lymphoma infiltration of the skin(1 cases), Lymphomatoid papulosis(3 cases), Extran- odal NK/T-cell lymphoma nasal type(1 cases ) and diffuse large B-cell lymphoma(2 cases). Only one positive reaction of EBER1/2 in situ hybridization was detected in Extranodal NK/T-celllymphoma, nasal type. TCR)'gene or IgH Ig gene colonel rearrangements were detected in 5/9 cases. Conclusion Lymphomas, especially T- cell lymphomas are the common diseases of cutaneous lymphoproliferative disorders. Mycosis Fungiodes is the most common cutaneous T cell lymphoma. The definite correlation of cutaneous lymphoproliferative disorders with Epstein-Barr virus was not he found. Both pathologic diagnosis and differentiate diagnosis of cutaneous lymphoproliferative disorders are challenging, and should be made with clinical information.