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胸腺原发性黏膜相关淋巴组织淋巴瘤2例及文献复习

常晓燕 陈杰 姜英 赵大春 凌庆 罗玉凤 冯瑞娥

常晓燕, 陈杰, 姜英, 赵大春, 凌庆, 罗玉凤, 冯瑞娥. 胸腺原发性黏膜相关淋巴组织淋巴瘤2例及文献复习[J]. 协和医学杂志, 2012, 3(1): 41-46. doi: 10.3969/j.issn.1674-9081.2012.01.010
引用本文: 常晓燕, 陈杰, 姜英, 赵大春, 凌庆, 罗玉凤, 冯瑞娥. 胸腺原发性黏膜相关淋巴组织淋巴瘤2例及文献复习[J]. 协和医学杂志, 2012, 3(1): 41-46. doi: 10.3969/j.issn.1674-9081.2012.01.010
Xiao-yan CHANG, Jie CHEN, Ying JIANG, Da-chun ZHAO, Qing LING, Yu-feng LUO, Rui-e FENG. Mucosa-associated Lymphoid Tissue Lymphoma of the Thymus: A Stuty of 2 Cases[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 41-46. doi: 10.3969/j.issn.1674-9081.2012.01.010
Citation: Xiao-yan CHANG, Jie CHEN, Ying JIANG, Da-chun ZHAO, Qing LING, Yu-feng LUO, Rui-e FENG. Mucosa-associated Lymphoid Tissue Lymphoma of the Thymus: A Stuty of 2 Cases[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 41-46. doi: 10.3969/j.issn.1674-9081.2012.01.010

胸腺原发性黏膜相关淋巴组织淋巴瘤2例及文献复习

doi: 10.3969/j.issn.1674-9081.2012.01.010
详细信息
    通讯作者:

    冯瑞娥 电话:010-65295970, E-mail:feng.ruie@yahoo.com.cn

  • 中图分类号: R733.4;R446.8

Mucosa-associated Lymphoid Tissue Lymphoma of the Thymus: A Stuty of 2 Cases

More Information
  • 摘要:   目的  探讨胸腺原发性黏膜相关淋巴组织淋巴瘤的临床病理特征、免疫组化和基因重排特点。  方法  复习2例胸腺原发性黏膜相关淋巴组织淋巴瘤的临床资料, 通过光镜观察、免疫组化染色(EnVision, 抗体包括CD3, CD20, CD79α, CD5, Bcl-2, Bcl-6, CD10, CD23, CyclinD1, AE1/AE3)、聚合酶链式反应(polymerase chain reaction, PCR)分析其临床病理特征、免疫组化和基因重排特点。  结果  2例患者均为汉族, 男女各1例, 年龄分别为57岁和53岁, 女性患者伴干燥综合征(Sjögren's syndrome, SS)及过敏性紫癜。术前CT、磁共振成像(magnetic resonance imaging, MRI)及术后大体检查均可见边界清楚的肿物, 内含小囊。镜下可见成片的小到中等大小弥漫淋巴细胞, 主要是中心细胞样细胞和/或单核样淋巴细胞, 偶见中心母细胞或免疫母细胞样细胞。小血管丰富, 血管周围可见较多浆细胞; 并可见多房囊肿, 囊壁被覆鳞状上皮细胞及柱状上皮细胞, 伴淋巴上皮病变, 囊壁Hassall小体增多并囊性退变, 内见粉染物; 存在反应性增生的淋巴滤泡, 周围脂肪组织内散见多处小结节。免疫组化显示CD20、CD79α和Bcl-2弥漫阳性, 囊肿上皮及赫氏小体(Hassall's corpuscle)AE1/AE3阳性, 上皮间可见CD20阳性的淋巴细胞浸润。PCR显示2例免疫球蛋白重链可变区(immunoglobulin heavy chain variable region, IgHV)基因重排阳性。2例患者均开胸行肿物及部分胸腺切除术, 术后分别随访27和6个月, 未见复发及转移。  结论  胸腺黏膜相关淋巴组织淋巴瘤少见, 具有明显特点:亚洲人多发, 伴有自身免疫性疾病, 大体及镜下可见多房囊肿, 血管周围可见明显的浆细胞分化, 缺乏凋亡抑制蛋白2-黏膜相关淋巴瘤转位基因1(API2-MALT1)融合基因等。手术切除为首选治疗, 临床结局良好。
  • 图  1  病例2, 肿瘤结节中可见一囊(箭头), 内含清亮液, 紧邻大囊可见一小囊(局部切片)

    图  2  低倍镜下胸腺呈多房囊肿, 囊壁及小结节几乎完全被淋巴组织取代

    图  3  淋巴上皮样病变, 淋巴细胞以中心细胞样细胞/单核样细胞为主, 细胞大小一致, 胞质较丰富且淡染

    图  4  CD20细胞膜勾勒清楚, 弥漫强阳性, 表面上皮内可见CD20阳性淋巴细胞浸润

    图  5  免疫球蛋白基因重排, IgHV可见异常条带, 显示Ig重排阳性

    IgHV:免疫球蛋白重链可变区

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出版历程
  • 收稿日期:  2011-10-27
  • 刊出日期:  2012-01-30

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