肺淋巴瘤样肉芽肿病的免疫表型及基因重排研究  

Pulmonary lymphomatoid granulomatosis： an immunohistochemical and gene rearrangement study

作　　者：冯瑞娥[1] 刘鸿瑞[1] 刘彤华[1] 陈杰[1] 凌庆[1] 师晓华[1] 钟定荣[1] 罗玉风[1] 曹金伶[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院病理科,100730

出　　处：《中华病理学杂志》2011年第7期460-464,共5页Chinese Journal of Pathology

摘　　要：目的观察肺淋巴瘤样肉芽肿病的细胞组成、免疫表型和分子生物学改变。方法回顾性分析北京协和医院9例肺淋巴瘤样肉芽肿病患者的临床病理情况。其中5例为开胸肺活检标本，3例为肺叶切除标本，1例尸检。标本经4％甲醛固定，石蜡包埋，常规切片，HE染色。免疫组织化学EnVision法染色（抗体包括CD20、CD3、CD56），原位杂交检测EB病毒，采用聚合酶链反应进行Ig和TCR基因重排检测。结果9例肺淋巴瘤样肉芽肿患者，年龄3～59岁，男：女=3：6。9例患者肺组织内病变分布均显示以血管为中心的淋巴细胞浸润为特点。免疫组织化学显示以CD3阳性的T淋巴细胞占绝对优势，散在不等数量的CD20阳性的B细胞，CD56均为阴性。8例行EB病毒原位杂交，4例阳性细胞数〈5％，3例〉20％，1例为15％。按照WHO的3级分级方法，I级为4例，Ⅱ级1例，Ⅲ级4例。6例行基因重排检测，3例显示有Ig基因重排阳性，其中，1例为Ⅱ级病变，2例为Ⅲ级病变。6例TCR重排检测均为阴性。随访时间0．5～6．5年不等，9例患者中3例死亡，2例存活，4例失访。结论部分肺淋巴瘤样肉芽肿病，特别是Ⅱ、Ⅲ级患者，有B淋巴细胞克隆性增生，提示其为淋巴瘤性病变。Objective To study the immunophenotype and gene rearrangement pattern of pulmonary lymphomatoid granulomatosis. Methods Nine cases of pulmonary lymphomatoid granulomatosis, included 5 cases of open lung biopsy, 3 cases of lobectomy specimen and 1 case of autopsy, were retrospectively analyzed by immunohistochemistry, in-situ hybridization for Epstein-Barr vilaas-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies. Results The age of patients ranged from 3 to 59 years. The male-to-female ratio was 3： 6. Histologically, all cases showed lymphocytic infiltration surrounding the blood vessels and in the perivascular areas. Most of these lymphoid cells expressed T-cell marker CD3. There were also variable numbers of CD20-positive B cells. The staining for CD56 was negative. According to the WHO classification, there were 4 cases of grade I , Ⅰ case of grade Ⅱ and 4 cases of grade Ⅲ lesions. Six cases had gene rearrangement studies performed and 3 of them demonstrated clonal immunoglobulin gene rearrangement （ including Ⅰ of the grade Ⅱ and 2 of the grade Ⅲ lesions）. No T-cell receptor gene rearrangement was detected. Conclusions Pulmonary lymphomatoid granulomatosis may represent a heterogeneous group of lymphoproliferative disorders. Some of the cases show B-cell immunophenotype and clonal immunoglobulin gene rearrangement, especially the grade Ⅱ and grade Ⅲ lesions. They are likely of lymphomatous nature.

关 键 词：肺肿瘤 淋巴瘤样肉芽肿病 基因重排 

分 类 号：R734.2[医药卫生—肿瘤]