肺黏膜相关B淋巴细胞淋巴瘤中BCL10蛋白表达和染色体易位的关系
High incidence of t(11;18)/API2-MALT1 and BCL10 aberrant nuclear expression in pulmonary mucosa-associated lymphoid tissue lymphomus
摘要目的 检测肺黏膜相关B淋巴细胞淋巴瘤(MALT淋巴瘤)中BCL10蛋白表达及目前报道的染色体易位的发生率.包括t(11;18)/AP12-MALTI、t(1;14)/BCLIO-lgH及t(14;18)/MALTI-IgH.探讨BCL10异常表达和染色体易位的关联程度.方法 收集复旦大学附属肿瘤医院病理科2000~2007年诊断的23例肺MALT淋巴瘤患者的病理组织蜡块,其中男19例,女4例,年龄27~84岁,平均55.8岁.用免疫组织化学EnVision法检测BCL10蛋白的表达,用荧光原位杂交(FISH)法分别检测API2-MALTI、BCL10、MALTl和IgH基因的异常,并对可联络到的10例病例进行随访.结果 23例中19例BCL10蛋白表达阳性,其中细胞质阳性9例,细胞核阳性者10例;用FISH方法榆测了全部病例,其中9例可检测到API2-MALTI融合基因.1例町能为BCL10-IgH融合,未发现IgH-MALT1基因异常.细胞核BCL10蛋白表达阳性的10例中,仅5例同时伴有基因异常;BCL10异常核表达与染色体易位无明显相关性(x2=0.306,P=0.685).有随访资料的10例患者全部生存(随访时间7~35个月),但治疗方式各异(单纯化疗、手术或手术加化疗).结论 肺MALT淋巴瘤患者组织内BCL10在细胞核中表达率较高,t(11;18)/AP12-MALT1融合基因是肺MALT淋巴瘤中最常见的染色体异常,而t(1;14)/BCL10-IgH和t(14;18)/MALT1-IgH在肺MALT淋巴瘤中不常见;核表达BCL10与发生染色体异常是瓦相独立的凶素,但这些特点对肺MALT淋巴瘤的诊断有一定的辅助价值,特别是对纤维支气管镜活检小标本组织的诊断更有帮助.
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abstractsObjective To detect the BCL10 expression and chromosomal translocations in pulmonary mucosa-associated lymphoid tissue (MALT) lymphomas, including t (11;18)/API2-MALT1 ; t(1 ;14)/IgH-BCLI0 and t(14;18)/MALTI-IgH, and to determine if aberrant nuclear BCL10 expression is related with chromosomal translocations. Methods Twenty-three cases of pulmonary MALT lymphomas were collected from Cancer Hospital of Fudan University. BCLIO was detected by immunohistoehemistry of EnVision method, and API2.-MALT1, BCLI0, MALTI, IgH chromosomal abnormalities were detected by fluorescent in situ hybridization (FISH) technique. Results BCLI0 was expressed in 82. 6% (19/23) of the pulmonary MALT lymphomas. Among those eases, 9 of 23 (39.1%) were expressed in the cytoplasm, and 10 of 23 (43.5%) were in the nucleus. In the FISH results, 9 cases (39.1%, 9/23) showed API2MALT1 fusion gene, 1 case with possible BCLI0-IgH abnormality, but none showed chromosomal abnormalities related with MALTI and IgH gene simultaneously. Among 10 BCL10 nuclear expressive cases only 5 harbored genetic abnormalities. There was no correlation between BCL10 abrerrant nuclear expression and chromosoma(translocations) (X2=0.306,P=0.685). Follow-up of 10 eases for a period of 7 to 35 months showed that all the patients were alive. Because different treatments applied in different patients(chemotherapy only, surgery with chemotherapy or surgery only), best available treatment could not be confirmed in this study. Conclusions t (11;18)/API2-MALT1 was the most common chromosomal abnormality in pulmonary MALT lymphomas, but t(1;14)/BCLI0-IgH and t(14; 18)/MALTl-lgH were rare. Pulmonary MALT lymphomas also had higher nuclear BCLI0 expression, which was not correlated with chromosomal abnormalities. As a result, BCLIO nuclear expression and cytogenetic aberration may be helpful in the diagnosis, especially for small biopsy specimens.
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