CAJ | 학술논문

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.
목적 관찰폐림파류양육아종병적세포조성、면역표형화분자생물학개변.방법 회고성분석북경협화의원9례폐림파류양육아종병환자적림상병리정황.기중5례위개흉폐활검표본,3례위폐협절제표본,1례시검.표본경4%갑철고정,석사포매,상규절편,HE염색.면역조직화학EnVision법염색(항체포괄CD20、CD3、CD56),원위잡교검측EB병독,채용취합매련반응진행Ig화TCR기인중배검측.결과 9례폐림파류양육아종환자,년령3～59세,남∶녀=3:6.9례환자폐조직내병변분포균현시이혈관위중심적림파세포침윤위특점.면역조직화학현시이CD3양성적T림파세포점절대우세,산재불등수량적CD20양성적B세포,CD56균위음성.8례행EB병독원위잡교,4례양성세포수<5%,3례>20%,1례위15%.안조WHO적3급분급방법,Ⅰ급위4례,Ⅱ급1례,Ⅲ급4례.6례행기인중배검측,3례현시유Ig기인중배양성,기중,1례위Ⅱ급병변,2례위Ⅲ급병변.6례TCR중배검측균위음성.수방시간0.5～6.5년불등,9례환자중3례사망,2례존활,4례실방.결론 부분폐림파류양육아종병,특별시Ⅱ、Ⅲ급환자,유B림파세포극륭성증생,제시기위림파류성병변.
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 virus-encoded RNA, immunoglobulin and T-cell receptor gene rearrangement studies. Results The 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 gradeⅠ, 1 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 1 of the grade Ⅱand 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 Ⅱand grade Ⅲ lesions. They are likely of lymphomatous nature.