肺淋巴管肌瘤病合并肺栓塞1例并文献复习

doi:10.3969/j.issn.1000-8535.2017.05.022

摘要/Abstract

摘要： 目的探讨肺淋巴管肌瘤病(PLAM)的临床特征,诊断和治疗,提高对PLAM的认识,合并顽固性低氧血症时需警惕并发肺动脉栓塞,预防漏诊及误诊。方法回顾分析1例确诊的PLAM合并肺栓塞患者病例资料,并结合相关文献进行复习。结果 PLAM是以弥漫性肺囊性病变为特征的一种罕见的缓慢进展的肿瘤性疾病,好发于育龄期女性,临床表现为呼吸困难、反复自发性气胸和乳糜胸。肺功能多表现为阻塞性或混合性通气功能障碍和弥散功能障碍,动脉血气显示低氧血症。高分辨CT(HRCT)示双肺弥漫性薄壁囊性改变。病理学检查示肺组织淋巴管增生和扩张,管外平滑肌细胞明显增生。诊断主要依靠高分辨CT、肺功能以及病理组织活检。血管内皮生长因子D是无创且可靠的血清学诊断标准。雷帕霉素抑制剂如西罗莫司科可有效治疗本病。结论育龄期妇女,如反复出现自发性气胸、活动后呼吸困难,胸部HRCT示双肺弥漫分布囊状改变,临床上应想到PLAM可能。随疾病的进展出现顽固性的呼吸困难、低氧血症不能纠正时,需考虑是否合并存在肺动脉栓塞的可能,CT肺动脉造影可确诊。

赖微微, 张挪富, 何梦璋, 顾莹莹. 肺淋巴管肌瘤病合并肺栓塞1例并文献复习[J]. 广州医药, 2017, 48(5): 84-88.

LAI Weiwei, ZHANG Nuofu, HE Mengzhang, GU Yingying. A case report of a patient with pulmonary lymphangiomyomatosis and pulmonary thromboembolism and literature reviews[J]. Guangzhou Medical Journal, 2017, 48(5): 84-88.

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