39例淋巴瘤样丘疹病临床病理学研究

摘要

Objective We studied the histological finding, immunophenotype and molecular biological characteristics for 39 cases of lymphomatoid papulosis. Materials and Methods Thirty-nine cases were collected from Guizhou Province. In addition to H. E, PAS and Giemsa staining, the immunohistochemical researches for CD20, CD45RO, CD4, CD8,CD30, CD15, ALK and EMA etc, and molecular biological studies for IgH and TCR were also performed. Results We classified all cases into three groups: type A of 33 cases, 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-cell population, including neoplastic T-cells were CD4 +/CD8- T-helper phenotype(12/21). CD 30 antigen intensively expressed in nearly all cases(36/39), in which,one case (type A) simultaneously expressed ALK and EMA. Ki-67 mean proliferation index for all cases was about 54%.Molecular biological studies demonstrated that four cases displayed TCRγ gene polyclonal rearrangement, in which, two cases respectively showed polyclonal and monoclonal rearrangement for Ig H. Conclusions Lymphomatoid papulosis predominantly affected adults/elderly. The male to female ratio was 4.6:1. Histological findings displayed that few number of large atypical lymphoid cells resemble Reed-Sternberg cells admixed in the background composed of a large number of inflammatory cells(type A) or the background composed of atypical lymphoid cells with cerebriform nuclei(type B).Morphorlogically, Type C was characteristic by nodular or sheet arranged large atypical lymphoid cells. In immunophenotype, CD30 antigen is nearly expressed in all cases except two cases of type B. Most of cases were CD4 +/CDg- phenotype and displayed the pan-T-cell antigen CD3.%目的对39例淋巴瘤样丘疹病进行了组织学、免疫表型和分子生物学特征研究.材料与方法 39例病例均来自贵州省各地.除H.E.,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例和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基因多克隆重排,其中,2例分别显示IgH多克隆和单克隆重排.结论淋巴瘤样丘疹病主要累及成人或老年人.男女之比是4.6:1.组织学特征表现为极少数H/R-S样的大的异型淋巴细胞混杂在大量炎性细胞背景(A型)中或主要为脑形核的异型淋巴细胞(B型).形态学上,C型以结节状或片状排列的大的异型淋巴细胞为特征.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细胞免疫表型,并且均表达全T-细胞抗原CD3.