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肺血管内淋巴瘤的临床及病理特征

葛莉 冯瑞娥 田欣伦 施举红 柳涛 徐作军 许文兵 宋伟 刘鸿瑞 朱元珏

葛莉, 冯瑞娥, 田欣伦, 施举红, 柳涛, 徐作军, 许文兵, 宋伟, 刘鸿瑞, 朱元珏. 肺血管内淋巴瘤的临床及病理特征[J]. 协和医学杂志, 2012, 3(1): 30-35. doi: 10.3969/j.issn.1674-9081.2012.01.008
引用本文: 葛莉, 冯瑞娥, 田欣伦, 施举红, 柳涛, 徐作军, 许文兵, 宋伟, 刘鸿瑞, 朱元珏. 肺血管内淋巴瘤的临床及病理特征[J]. 协和医学杂志, 2012, 3(1): 30-35. doi: 10.3969/j.issn.1674-9081.2012.01.008
Li GE, Rui-e FENG, Xin-lun TIAN, Ju-hong SHI, Tao LIU, Zuo-jun XU, Wen-bing XU, Wei SONG, Hong-rui LIU, Yuan-jue ZHU. Clinical and Pathological Features of Pulmonary Intravascular Lymphoma[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 30-35. doi: 10.3969/j.issn.1674-9081.2012.01.008
Citation: Li GE, Rui-e FENG, Xin-lun TIAN, Ju-hong SHI, Tao LIU, Zuo-jun XU, Wen-bing XU, Wei SONG, Hong-rui LIU, Yuan-jue ZHU. Clinical and Pathological Features of Pulmonary Intravascular Lymphoma[J]. Medical Journal of Peking Union Medical College Hospital, 2012, 3(1): 30-35. doi: 10.3969/j.issn.1674-9081.2012.01.008

肺血管内淋巴瘤的临床及病理特征

doi: 10.3969/j.issn.1674-9081.2012.01.008
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

Clinical and Pathological Features of Pulmonary Intravascular Lymphoma

More Information
  • 摘要:   目的  探讨肺血管内淋巴瘤的临床及病理特征。  方法  回顾性观察及总结了2008年3月至2011年6月北京协和医院经肺活检病理诊断的5例肺血管内淋巴瘤患者的临床、胸部电子计算机断层摄影(computed tomography, CT)及病理资料, 并复习文献。活检标本经10%中性福尔马林固定, 常规石蜡切片, HE及免疫组化染色。  结果  5例肺血管内淋巴瘤患者中, 男3例, 女2例; 年龄36~59岁, 中位年龄45岁。临床症状主要为发热(5/5)、体重减轻(5/5)、咳嗽(4/5)、乏力(3/5)和气短(2/5)等; 5例患者血清乳酸脱氢酶均有不同程度升高(316~1025 U/L)。肺功能以弥漫性功能障碍为主。肺部CT:3例表现为双肺多发磨玻璃及实变影, 1例为多发结节影、胸膜下楔形实变影及支气管血管束增粗, 1例表现为右肺下叶实变影。病理组织学:肺血管内淋巴瘤主要表现为单个或小簇淋巴瘤细胞分布于狭小的肺泡间隔毛细血管腔内, 保留肺泡结构, 伴有肺泡上皮增生, 其病变微小, 易漏诊。1例肿瘤细胞形成瘤栓伴肺梗死。免疫组化显示3例为B细胞性, 2例为T细胞性。随访20 d~6个月(平均3.17个月), 3例B细胞性血管内淋巴瘤患者均行R-CHOP方案化疗, 2例化疗后病情平稳存活, 1例化疗6程后病情进展, 更换Hyper-CVAD方案化疗后病情平稳存活; 2例T细胞性血管内淋巴瘤患者, 1例失访, 1例经CHOP方案化疗效果不佳, 于诊断后20 d死于消化道出血和呼吸衰竭。  结论  肺血管内淋巴瘤非常少见, 常见于中老年人, 其临床症状缺乏特异性, 胸部CT常表现为弥漫性间质性病变。病理组织学显示, 淋巴瘤细胞主要分布于狭小的肺泡间隔毛细血管腔内, 其病变微小、易漏诊, 免疫组化有助于其诊断。
  • 图  1  活检肺组织肺泡间隔略增宽, 肺泡间隔毛细血管腔内可见单个(小箭头)或几个成簇(大箭头)的淋巴瘤细胞(HE 10×20)

    图  2  CD20免疫组织化学染色示肺间隔毛细血管腔内淋巴瘤细胞染色阳性(HE 10×10)

    图  3  CD34免疫组织化学染色示血管内皮细胞染色阳性, 淋巴瘤细胞位于内皮细胞内(HE 10×10)

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出版历程
  • 收稿日期:  2011-10-25
  • 刊出日期:  2012-01-30

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