原发皮肤淋巴瘤临床病理学研究

Study on Clinicopathology in Primary Cutaneous Lymphoma

【目的】进一步探讨原发皮肤淋巴瘤(primary cutaneous lymphoma,PCL)的临床病理、免疫组化和分子生物学特点,着重研究和总结一些PCL的特殊类型。【方法】筛选1983年1月至2005年12月间北京铁路总医院诊断为PCL者40例。收集40例的主要临床资料和库存组织蜡块,常规HE染色和免疫组化标记,并选出25例做TCRβ/γ和IgHFr2A/Fr3A基因重排检测。对全部病例进行追踪随访。【结果】40例平均年龄(41.53±20.14)岁。老年男性略占优势。肿瘤病变以躯干和上肢首发为主。临床表现以皮下结节为主。光镜下,一般类型PCL瘤细胞分布具有结外同类型T、B淋巴瘤细胞的特点,特殊类型如原发皮肤CD30阳性/阴性大T细胞淋巴瘤、原发皮肤弥漫大B细胞淋巴瘤、原发皮肤淋巴母细胞淋巴瘤和皮下脂膜炎样T细胞淋巴瘤具有独特特点。免疫组化和基因重排检测对分型有帮助。【结论】①PCL并不罕见,本组原发皮肤T细胞淋巴瘤共28例(70%);原发皮肤B细胞淋巴瘤共9例(22.5%),以原发皮肤T细胞淋巴瘤占优势。②PCL有其不同于结内或结外其他部位淋巴瘤的独特亚型,建议采用综合EORTC分类与WHO 2001淋巴造血组织分类的修订分类。③CD30的表达与否为原发皮肤大T细胞淋巴瘤最主要的预后指标,CD30阳性者预后好,CD30阴性者预后差。④免疫组化最好两种以上抗体协同使用。⑤基因重排检测对于PCL的早期和交界性病变诊断有重要参考意义。

[Objective] To investigate the clinicopathologic, immunohistochemical and molecular biological features, and to emphasize in studing and summarizing special kinds of primary cutaneous lymphoma. [Methods]There were 40 cases which were previously diagnosed as primary cutaneous lymphoma from 1983 to 2001 in Beijing Railway Hospital （including clinical data and tissue blocks）. All cases were investigated by using HE and immunohistochemical methods, and TCRβ/γ and IgHFr2A / Fr3A gene assay were employed in 25 cases. The follow-up information was obtained for all cases. [Results]Median age was （41.53 ± 20.14） years（range 2-73 years）. The lesions showed in generalize, especially in trunk and upper limb. Nodule was much more than others. Some various subtypes （for example, PCCD30 positive/negative large-cell lymphoma, primary cutaneous diffuse large B cell lymphoma, primary cutaneous blastoma and subcutaneous panniculitic T cell lymphoma） had different clinical and histological features. Immunohistochemical method and gene rearrangement were helpful to diagnosis. [Conclusion]1.Primary cutaneous lymphoma was not rare, and most of them were primary cutaneous T-cell lymphoma. 2.There were some special subtypes in primary cutaneous lymphoma that were different from nodal or extranodal lymphoma （except skin）. The revised classification （integration of EORTC and WHO2001） should be used.3.CD30 was a primary sign of prognosis of primary cutaneous large-cell lymphoma. CD30-positive was good, whereas poor. 4.Two or more antibodies had better use in diagnosis. 5.Gene rearrangement assay was helpful in distinguishing from early or borderline pathological changes of primary cutaneous lymphoma.