北京大学学报（医学版）Issue(5)：865-869,5.DOI:10.3969/j.issn.1671-167X.2015.05.026
肺毛细血管瘤病1例及文献回顾
Pulmonary capillary hemangiomatosis:a case report and literature review
熊焰 1李晓霞 1牟向东 2李东 1王颖 1李挺1
作者信息
- 1. 北京大学第一医院 病理科，北京 100034
- 2. 北京大学第一医院 呼吸内科，北京 100034
- 折叠
摘要
Abstract
We reported a case of pulmonary capillary hemangiomatosis (PCH) and introduced its diag-nosis, differential diagnosis, pathogenesis and development of treatment based on the review of Dana Point 2008 Classification of Pulmonary Hypertensiona and current literatures .A 43-year-old female presented progressive dyspnea, elevated pulmonary arterial pressures and CT pulmonary angiography (CTPA) imaging of main pulmonary arterial enlargement and wide spread ill -defined centrilobular nodules of ground-glass opacity.Her histologic features were proliferation of capillary channels within alveolar walls as well as muscularization of arterioles and medial hypertrophy of muscular pulmonary arteries.The treatment with diuretics and warfarin was used promptly , but unfortunately was ineffective. The patient died three months after diagnosis .PCH is a very rare vascular disease with poor prognosis . The diagnosis of PCH rests on the integration of clinical and radiographic information with pathologic fea -tures, however pathology is the most reliable means .Because clinical symptoms, imaging and histological features of pulmonary veno-occlusive disease (PVOD) and PCH broadly overlap, differential diagnosis should be made carefully.Among the various pathologic features proliferation of capillaries within alveolar walls is the key point for diagnosing PCH , which is also the most critical criteria for differentiating PCH from PVOD.So far the only definitive treatment for PCH is lung transplantation , without which the pa-tient will die several months after diagnosis .关键词
肺毛细血管瘤病/肺静脉闭塞性疾病/肺动脉高压Key words
Pulmonary capillary hemangiomatosis/Pulmonary veno-occlusive disease/Pulmonary arte-rial hypertension分类
医药卫生引用本文复制引用
熊焰,李晓霞,牟向东,李东,王颖,李挺..肺毛细血管瘤病1例及文献回顾[J].北京大学学报（医学版）,2015,(5):865-869,5.
