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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。
To study the clinical features, pathological features, immunophenotypes, molecular biological features and prognosis of pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma and to summarize the clinical and pathological features of the lung MALT lymphoma. Methods Twenty-three cases of primary MALT lymphoma were studied by clinical data summary, pathological morphological observation and immunophenotypic analysis. Clonal rearrangements of Ig H gene were performed in 5 of them and 4 cases of fluorescent Bit hybridization (FISH) MALT1 gene detection. Results Twenty-three of 23 primary MALT lymphoma patients were asymptomatic and found pulmonary lesions on physical examination. The rest were cough and chest pain. Imaging showed lung shadow or solid shadow, 19 lesions located in the right lung. Pathological features of 5 cases of tumor was nodular, 18 cases of diffuse growth, tumor and lung tissue can be seen at the junction of island-like or beaded-like structure. The tumor consisted of three kinds of morphological cells: centrocyte-like cells, mononuclear B cells and small lymphocyte-like cells. Among them, 13 cases were accompanied by differentiation of plasma cells, and both lymphoepithelial lesions and follicular infiltration were observed. Immunohistochemical staining revealed that the tumor cells were CD20 (+) and Ki-67 index of 5% -20%. Eight cases of Igκ and Igλ showed that the tumor cells were monoclonal B cells. In molecular biology, single clonal bands were detected by IgH clonal rearrangements, and MALT1 gene was detected by FISH in 2 of 4 cases. Four patients in this group were lost to follow-up, and the remaining 19 patients were followed up for 3 months to 6 years and 5 months, all of whom survived. Five of the 5 patients had local tumor recurrence. Eight patients underwent tumor resection only, and 11 patients underwent surgical resection and received chemotherapy after operation. Conclusions There are asymptomatic or mild symptoms in lung primary MALT lymphoma. The lungs are lumps or shadows on imaging. The tissue morphology is nodular or diffuse mononuclear small lymphocyte infiltration. Beaded-like structure, was B-cell immunophenotype and no other small B cell lymphoma phenotype, IgH clonality was detected by monoclonal antibody, FISH MISH1 gene was found to be broken. The prognosis of this disease is better, may be local recurrence.