【摘要】 研究肺原发性黏膜相关淋巴组织(MALT)淋巴瘤的临床表现、病理学形态特征、免疫表型、分子生物学特点及预后情况,总结肺原发性MALT淋巴瘤的临床及病理学特点。方法通过临床资料总结、病理学形态观察、免疫表型分析,对23例肺原发性MALT淋巴瘤病例进行研究,并且对其中5例行Ig H基因克隆性重排检测,4例行荧光原位杂交(FISH)MALT1基因检测。结果 23例肺原发性MALT淋巴瘤中12例无症状,于体检时发现肺部病变,其余表现为咳嗽、胸痛等。影像学均表现为肺部肿块影或实变影,19例病变位于右肺。病理学形态特点,5例肿瘤呈结节状,18例弥漫性生长,肿瘤与肺组织交界处均可见孤岛样或串珠样结构。肿瘤由3种形态细胞构成:中心细胞样细胞、单核样B细胞、小淋巴细胞样细胞,其中13例伴浆细胞分化,均可见到淋巴上皮病变及滤泡殖入现象。免疫组化染色,肿瘤细胞CD20(+),Ki-67指数5%~20%,8例Igκ、Igλ显示肿瘤细胞为单克隆性B细胞。分子生物学方面,Ig H克隆性重排检测均检测到单克隆条带,4例中2例FISH检测出MALT1基因断裂。本组4例失访,其余19例随访3个月~6年5个月,全部存活,其中5例可疑肿瘤局部复发。8例仅行肿瘤局部切除,11例行手术切除并于术后行化疗。结论肺原发性MALT淋巴瘤无症状或仅有轻微症状,影像学显示肺部肿块或阴影,组织形态表现为结节状或弥漫性单核样小淋巴细胞浸润,周边伴特征性孤岛样或串珠样结构,呈B细胞免疫表型并无其他小B细胞性淋巴瘤的表型特征,Ig H克隆性检测呈单克隆,FISH检测可见MALT1基因断裂。本病预后较好,可局部复发。更多还原

【Abstract】 Objective To investigate the clinical manifestation, morphological and immunophenotypic features,molecular biological characteristics,and prognosis of primary pulmonary MALT lymphoma,and then to summarize its clinical and pathological characteristics. Methods A total of 23 cases of primary pulmonary MALT lymphoma were collected,and clinical data, morphological features and immunophenotype were analyzed. 5 cases were studied by polymerase chain reaction( PCR) to detect B cell clonality. 4 cases were studied by interphase fluorescence in situ hybridization( FISH) to detect whether MALT1 gene was broken or not. Results 12 of 23 patients with primary pulmonary MALT lymphoma were asymptomatic,and pulmonary lesions observed in X-ray examination. Other patients only had mild symptoms,such as cough,chest pain,and so on. Imaging showed that all cases manifested as a pulmonary mass or consolidation. Tumor occured predominantly in the right lung. In our study group,19 of 23 patients only had right lung involvement. Histologically, surgical specimens showed that neoplastic lymphocytes infiltrated in nodular or diffuse patterns. Tumor cells infiltrated in the lung tissue,formed a characteristic structure in the periphery:"isolated island"or"string-of-beads". Tumors were composed of three types of cells: centrocyte-like cells, lymphocytoid cells, and monocytoid B cells. More or less plasma cell differentiation was observed in 13 of 23 cases,and lymphoepithelial lesions and follicular involvement were noted in all the cases. As to immunophenotype,the neoplastic cells were positive for CD20,and Ki-67 index was 5 to 20 percentage. 8 of 13( 61. 54%) which stained for kappa or lambda showed neoplastic cells were monoclonal B cells. B-cell clonality was detected by PCR in five samples with monoclonal bands. FISH test for MALT1 breakup gene was positive in 2 of 4 cases. In this group of primary pulmonary MALT lymphoma,4 cases were lost to follow-up. The other 19 cases were followed from 3 months to 6 years and 5 months. All patients were alive,but 5patients seemed to relapsed locally in the lung. In the aspect of therapy,8 of 19 patients who were followed up only had tumor removed surgically,other 11 patients underwent chemotherapy after the surgery. Conclusion Patients with primary pulmonary MALT lymphoma are often asymptomatic,or only have mild symptoms in clinical manifestation. In imageological examination,patients often manifest as a mass or consolidation in the lung. All tumors showed that lymphocytic infiltration presents in nodular or diffuse patterns. Tumor cells infiltrate the lung tissues,and then form a characteristic structure: " isolated islands"or"string-of-beads". Neoplastic cells express B cell markers,without other small B-cell lymphocytic lymphoma’s features. B cell clonality of PCR always can be detected with monoclonal bands,and MALT1 gene breakup in FISH can be positive. Primary pulmonary MALT lymphoma has a good prognosis,but tumor can relapse locally.更多还原