肺淋巴管平滑肌瘤病合并肺高血压1例Pulmonary lymphangioleiomyomatosis complicated with pulmonary hypertension:a case report
张洁,卢祥婷,戴海龙
摘要(Abstract)：
肺淋巴管平滑肌瘤病（PLAM）是一种罕见的低级别、破坏性、转移性肿瘤，以肺部弥漫性囊性病变为特征的多系统疾病，主要发生于育龄期女性。肺部疾患中出现肺高血压（PH）往往提示预后不良，而PLAM合并PH的病例报道更是较为少见。本病例报道1例45岁女性患者，以反复气促为主要表现，完善右心导管检查、超声心动图、胸部CT、肺功能、血管内皮细胞生长因子-D(VEGF-D)等相关检查，诊断为PLAM合并PH。本病例报道对于PLAM合并PH的患者及早诊断并采取相应措施具有重要的临床意义。
关键词(KeyWords)： 肺淋巴管平滑肌瘤病;肺高血压;血管内皮细胞生长因子-D
基金项目(Foundation): 国家科技重大专项（2024ZD0526701）;; 国家自然科学基金项目（82060018、81700438）;; 云南省科技项目（202301AY070001-300、202405AJ310005-17）;; 云南省创新团队项目（202505AS350027）
作者(Author): 张洁,卢祥婷,戴海龙
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