皮肤淋巴瘤样丘疹病39例临床病理学研究

Pathologic study on lymphomatoid papulosis a report of 39 cases

目的:研究皮肤淋巴瘤样丘疹病的组织学、免疫表型和分子生物学特征。方法:39例病例均来自贵州省各地。除HE、PAS和Giemsa染色外,进行了CD20、CD45RO、CD4、CD8、CD15、CD30、ALK及EMA等免疫组织化学研究,以及IgH和TCR分子生物学研究。结果:全部病例分成3型:A型33例,B型2例,C型4例。在大多数病例(31/33),肿瘤细胞特别是大细胞表达CD3抗原。大多数T细胞组群,包括瘤样T细胞表达CD4+/CD8-辅助T表型(12/21)。几乎所有病例(36/39)都表达CD30抗原,其中1例(A型)同时表达ALK和EMA。Ki-67增殖指数平均为54%。4例表达TCRγ基因单克隆重排。结论:淋巴瘤样丘疹病主要见于成人或老年人。男女之比是4.6∶1。组织学特征表现为极少数H/R-S样的大异形淋巴细胞混杂在大量炎性细胞背景(A型)中或主要为脑型核的异形淋巴细胞(B型)。C型以结节或片状排列的大异形淋巴细胞为特征。免疫表型上,除2例B型外,其余病例均表达CD30抗原。大多数病例为CD4+/CD8-辅助T表型,并且均表达全T-细胞抗原CD3。

OBJECTIVE：To study the histological finding, immunophenotype and molecular biological characteristics of lymphomatoid papulosis. METHODS： Thirty-nine patients form Guizhou Povince were investigated. Inaddition to HE. PAS and Giemsa staining, the immunohistochemical researches for CD20 CD45 RO, CD4, CD8 , CD30, CD15 , ALK and EMA etc,and molecular biological studies for TCR were performed. RESULTS： All cases were classified into three groups：Type A of 33 ca ses. Type B of 2 cases and Type C of 4 cases. In most cases （31/33）, the tumor cells, especially large cells, expressed CD3 antigen. Most T-cells population, including neoplastic T-cells. were CD4^＋ CD8 T-helper phenotype （ 12/21 ）. CD30 antigen intensively expressed in nearly all cases （36/39）. in which, one case （Type A） stimultaneously expressed ALK and EMA. Ki-67 mean proliferation index for all cases was about 54 %. Molecular biological studies demonstrated that four cases displayed TCR gene monoclonal rearrangement for IgH. CONSLUSION： Lyphomatoid papulosis predominantly affects adults/elderly. The male to female ratio is 4.6 ： 1. Histological findings display that few number of atypical lymphoid cells resemble Reed-Sterberg cells admix in the backgroud composed of a large number of inflammatory cells （Type A） or the backgroud composed of atypical lymphoid cells with cerebriform nuclei （Type B）. Mophorlogical, Type C is characteristic of nodular or sheet arranged large atypical cells. In immunophenotype, CD30 antigen is nearly expressed in all cases except two cases of Type B. Most of cases are CD4^＋ / CD8 phenotype and display the pan-T-cell antigen CD3.