Clinical primary pulmonary hypertension: three pathologic types.
Clinical primary pulmonary hypertension: three pathologic types.
复制标题
临床原发性肺动脉高压：三种病理类型。
作者：
W. Edwards;J. Edwards
临床上，有一群肺动脉压升高的患者，其根本原因尚不清楚。肺动脉楔压不升高。对于此类病例，临床上可指定原发性肺动脉高压。从原发性肺动脉高压的临床分类来看，出现了三种不同的病理实体，即1)丛源性肺动脉病，2)复发性肺血栓栓塞，和3)肺静脉闭塞性疾病。丛原型最初的特征是肺动脉血管收缩伴内侧肥厚。继发性增殖性内膜病变，包括丛状病变，出现。复发性肺血栓栓塞的特征是存在不同年龄的动脉血栓，累及微小的肺动脉。血栓本质上可能是栓塞的，也可能是原位形成的。肺静脉闭塞性疾病的特征是肺静脉和小静脉的阻塞性病变。三种病理类型的临床表现可能非常相似，以至于最终的诊断取决于对活检或尸检获得的组织进行肺部组织学检查。
Clinically, there is a group of patients with elevated pulmonary arterial pressure in whom the underlying cause is not apparent. The pulmonary arterial wedge pressure is not elevated. For such cases, the designation of primary pulmonary hypertension may be made clinically. From the clinical categorization of primary pulmonary hypertension, three distinct pathologic entities emerge, namely 1) plexogenic pulmonary arteriopathy, 2) recurrent pulmonary thromboembolism, and 3) pulmonary veno-occlusive disease. The plexogenic type is characterized initially by pulmonary arterial vasoconstriction with medial hypertrophy. Secondary proliferative intimal lesions, including the plexiform lesion, develop. Recurrent pulmonary thromboembolism is characterized by the presence of arterial thrombi of varying ages involving the microscopic-sized pulmonary arteries. Thrombi may be embolic in nature or may develop in situ. Pulmonary veno-occlusive disease is characterized by obstructive lesions of pulmonary veins and venules. The clinical presentation of the three pathologic types may be so similar that definitive diagnosis depends upon histologic examination of the lung from tissue obtained either by biopsy or at necropsy.