儿童特发性肺含铁血黄素沉着症CT表现、病理特点  

CT findings and pathological features of idiopathic pulmonary hemosiderosis in children

作　　者：叶丽芳[1] 曾欣[2] 陈蒙蒙 邓宇[1] 曾庆思[1] 蒙秋华[1] YE Lifang;ZENG Xin;CHEN Mengmeng;DENG Yu;ZENG Qingsi;MENG Qiuhua(Department of Radiology,the First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510120,China;Department of Pathology,the First Affiliated Hospital of Guangzhou Medical University,Guangzhou 510120,China;Department of Thoracic Surgery,the First Affiliated Hospital of Sun Yat-sen University,Guangzhou 510080,China)

机构地区：[1]广州医科大学附属第一医院放射科,广东广州510120 [2]广州医科大学附属第一医院病理科,广东广州510120 [3]中山大学第一附属医院胸外科,广东广州510080

出　　处：《实用放射学杂志》2022年第6期980-982,1011,共4页Journal of Practical Radiology

摘　　要：目的探讨儿童特发性肺含铁血黄素沉着症(IPH)的CT表现及病理特点.方法回顾性分析经临床与病理确诊IPH的患儿33例,分析其CT表现及病理特点.结果33例患儿中,13例处于急性肺出血期,CT表现为肺野透亮度不均匀减低,呈磨玻璃样改变伴弥漫分布斑点状、粟粒状磨玻璃密度影;病理为弥漫性肺泡毛细血管出血,肺泡腔内大量红细胞及渗出液,含铁血黄素巨噬细胞聚集.12例处于慢性出血期,CT表现为两肺广泛分布的细网格状影及散在微小结节影;病理为肺间质内有大量含铁血黄素沉着,纤维组织增生,肺泡壁及小叶间隔增厚和含铁的纤维结节形成.8例处于慢性迁延后遗期,CT表现为两肺弥漫性间质性纤维化改变;病理为肺间质纤维化、肺泡过度充气.结论IPH各期CT表现和病理表现具有一定特点,当CT表现为弥漫性肺泡出血,同时合并细网格影、结节影,病理出现含铁血黄素结节时,要考虑IPH.Objective To explore the CT findings and pathological features of idiopathic pulmonary hemosiderosis(IPH)in children.Methods The CT findings and pathological features of 33 children with IPH confirmed by clinic and pathology were analyzed retrospectively.Results Of the 33 patients,13 were in the acute pulmonary hemorrhage stage,and CT showed inhomogeneous ground glass changes with diffuse distribution of speckle and miliary opacities,with pathologic changes including diffuse alveolar capillary hemorrhage,with a large number of red blood cells and exudate and hemosiderin macrophages in alveolar.12 were in the chronic hemorrhage stage,and CT showed wide distribution of reticular pattern and scattered nodules,with pathologic changes including massive hemosiderin deposition in lung interstitium,proliferation of fibrous tissue,thickening of alveolar wall and interlobular septum,and formation of iron containing fibrous nodules.8 were in the chronic sequclac stage,and CT showed diffuse interstitial fibrosis in bilateral lungs,with pathologic changes of pulmonary interstitial fibrosis and alvcolar hyperinflation.Conclusion The CT findings and pathological features of IPH at each stage have certain characteristics.IPH should be taken into consideration when the diffuse alveolar hemorrhage combined with reticulations and nodules on CT and hemosiderin nodules in pathology are observed.

关 键 词：特发性肺含铁血黄素沉着症 计算机体层成像 病理学 

分 类 号：R563.7[医药卫生—呼吸系统] R814.42[医药卫生—内科学] R446.8[医药卫生—临床医学]