Molecular and Clinicopathologic Significance of Gene Alteration Associated with MALT Lymphoma

与 MALT 淋巴瘤相关的基因改变的分子和临床病理意义

• 批准号: 13670185
• 负责人: INAGAKI Hiroshi
• 金额: $ 2.18万
• 依托单位: Nagoya City University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2001
• 资助国家: 日本
• 起止时间: 2001 至 2002
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-13670185/
• 关键词: MALT lymphoma; API2-MALT1 fusion; multiplex RT-PCR; clinicopathologic study; lung; thymus; skin; BCL10

API2-MALT1 fusion gene, cloned from t(11;18)(q21;q21) chromosomal translocation, is a gene abnormality specific to extranodal marginal zone B-cell lymphoma of mucosal-associated lymphoid tissue (MALT). However, the clinicopathologic significance of this chimeric gene remains to be clarified. We have developed a very sensitive multiplex RT-PCR assay for the API2-MALT1 fusion transcript using paraffin materials as a source of RNA, and enabled a large cohort study on this gene abnormality. Our data of a large series of pulmonary MALT lymphoma suggest that the API2-MALT1 fusion may be a causative gene abnormality unrelated to autoimmune disease. In addition, this alteration may define a homogeneous MALT lymphoma subtype that is clinically more indolent and histologically more "typical". Our observation that API2-MALT1 fusion is independence of autoimmune disease is further supported by the data obtained from the study of thymic MALT lymphoma. Aberrant nuclear BCL10 expression may have a possible role as a tool to screen for this API2-MALT1 fusion in pulmonary MALT lymphoma, but this is not the case with cutaneous MALT lymphoma. Case of rare esophageal and ureteral MALT lymphomas have been reported.

API 2-MALT 1融合基因是从t（11;18）（q21;q21）染色体易位中克隆到的一种粘膜相关淋巴组织（MALT）结边缘区B细胞淋巴瘤特异性基因异常。然而，这种嵌合基因的临床病理意义仍有待澄清。我们已经开发了一个非常敏感的多重RT-PCR检测API 2-MALT 1融合转录物使用石蜡材料作为RNA的来源，并使这种基因异常的大型队列研究。我们的一个大系列的肺MALT淋巴瘤的数据表明，API 2-MALT 1融合可能是一个致病基因异常无关的自身免疫性疾病。此外，这种改变可以定义一个同质的MALT淋巴瘤亚型，临床上更惰性和组织学上更“典型”。从胸腺MALT淋巴瘤研究中获得的数据进一步支持了我们的观察，即API 2-MALT 1融合不依赖于自身免疫性疾病。异常的核BCL 10表达可能作为一种工具，以筛选这种API 2-MALT 1融合在肺MALT淋巴瘤，但这不是皮肤MALT淋巴瘤的情况。罕见的食管和输尿管MALT淋巴瘤的情况下，已报告。

项目成果

Okabe M, Inagaki H et al.: "ApI2-MALT1 fusion defines a distinctive clinicopathologic subtype in pulmonary extranodal margined zone B-cell lymphoma of mucosa-associated lymphoid tissue"Am J Pathol. 162. 1113-1122 (2003)

Okabe M、Inagaki H 等人：“ApI2-MALT1 融合定义了粘膜相关淋巴组织的肺结外边缘区 B 细胞淋巴瘤的独特临床病理学亚型”Am J Pathol。

Lic, Inagaki H et al.: "Primary coetaneous marginal zone B-cell lymphoma : A molecular and clinicopathologic shady of 24 Asian cases"Am J Surg Pathol. (発表予定).

Lic、Inagaki H 等人：“原发性并发边缘区 B 细胞淋巴瘤：24 例亚洲病例的分子和临床病理学阴影”Am J Surg Pathol（即将发表）。

Hosaka S, Inagaki H et al.: "A case of primary low grade mucosa-associated lymphoid tissue (MALT) lymphoma of the oesophagus"Gut. 51. 281-284 (2002)

Hosaka S、Inagaki H 等：“食管原发性低度粘膜相关淋巴组织 (MALT) 淋巴瘤一例”Gut。

Hara M, Inagaki H et al.: "Primary ureteral mucosa-associated lymphoid tissue (MALT) lymphoma"Radiat Med. 20. 41-44 (2002)

Hara M、Inagaki H 等：“原发性输尿管粘膜相关淋巴组织 (MALT) 淋巴瘤”Radiat Med。

Inagaki H, Chan JKC, Ng JWM, Okabe M, Yoshino T, Okamoto M, Ogawa H, Matsushita H, Yokose T, Matsuno Y, Nakamura N, Nagasaka T, Ueda R, Eimoto T, and Nakamura S.: "Primary Thymic Extranodal Marginal Zone B-cell Lymphoma of Mucosa-Associated Lymphoid Tissu

Inagaki H、Chan JKC、Ng JWM、Okabe M、Yoshino T、Okamoto M、Okawa H、Matsushita H、Yokose T、Matsuno Y、Nakamura N、Nagasaka T、Ueda R、Eimoto T 和 Nakamura S.：“初级胸腺