Erbersdobler A, Niendorf A
Institute of Pathology, University Hospital Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany.
Histopathology. 2002 Jan;40(1):88-91. doi: 10.1046/j.1365-2559.2002.01322.x.
We investigated a case of pulmonary capillary haemangiomatosis, a rare condition, to determine the extent of the pathological changes within the lungs. Systematic histological sampling has not previously been performed in this condition.
A 52-year-old woman with a history of ischaemic cardiomyopathy suffered from repeated respiratory infections, which were attributed to chronic pulmonary congestion. She died suddenly of fulminant pulmonary thromboembolism. An autopsy was performed and lung tissue was sampled at multiple sites. Beside passive congestion, the lungs showed well-circumscribed areas containing proliferations of small capillaries infiltrating the pulmonary septa and the walls of otherwise normal blood vessels and bronchi. The most severely affected areas were found to be in the periphery of both lower lobes. A diagnosis of pulmonary capillary haemangiomatosis was made.
This is the first case of pulmonary capillary haemangiomatosis in which systematic histological sampling has been performed. Mapping of lesions disclosed the multifocal distribution of pulmonary capillary haemangiomatosis in this patient.
我们研究了一例肺毛细血管瘤病（一种罕见疾病），以确定肺部病理变化的范围。此前尚未对这种疾病进行过系统的组织学采样。
一名有缺血性心肌病病史的52岁女性反复发生呼吸道感染，归因于慢性肺淤血。她因暴发性肺血栓栓塞症突然死亡。进行了尸检，并在多个部位采集了肺组织样本。除了被动性淤血外，肺部可见边界清晰的区域，其中小毛细血管增生，浸润肺间隔以及原本正常的血管和支气管壁。发现受影响最严重的区域位于两下叶周边。诊断为肺毛细血管瘤病。
这是首例进行了系统组织学采样的肺毛细血管瘤病病例。病变图谱显示该患者的肺毛细血管瘤病呈多灶性分布。
