PULMONARY TUMOR THROMBOTIC MICROANGIOPATHY WITH PULMONARY-HYPERTENSION
PULMONARY TUMOR THROMBOTIC MICROANGIOPATHY WITH PULMONARY-HYPERTENSION
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DOI：
10.1002/1097-0142(19900801)66:3
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发表时间：
1990-08-01
期刊：
影响因子：
6.2
通讯作者：
OTTO, HF
中科院分区：
文献类型：
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作者：
VONHERBAY, A;ILLES, A;OTTO, HF
肺肿瘤血栓性微血管病变是转移性肺癌患者肺小动脉和小动脉纤维细胞内膜增生的特征。对630例(3.3%)连续尸检确诊的21例肿瘤的形态特征，包括癌前病变进行了研究。21例患者中19例为腺癌，11例为胃癌。致病事件始于微小的肿瘤细胞栓塞症。肿瘤栓子不会阻塞受影响的血管，但会导致局部凝血激活和纤维细胞内膜增殖，从而导致狭窄或闭塞。血流动力学方面，血管阻力增加会导致肺动脉高压。在3名患者中，转移癌生前未知，诊断为来源不明的肺动脉高压。因此，在原发性肺动脉高压的鉴别诊断中应考虑肺肿瘤血栓性微血管病变，特别是在患有急性或亚急性肺源性心脏病的知名癌症患者中。
Pulmonary tumor thrombotic microangiopathy is characterized by fibrocellular intimal proliferation of small pulmonary arteries and arterioles in patients with metastastic carcinoma. Its morphologic features, including precursor lesions, were studied in 21 patients diagnosed in 630 consecutive autopsy cases with carcinoma (3.3%). Nineteen of 21 patients had adenocarcinoma and 11 of these 19 patients had gastric carcinoma. The pathogenetic events start with microscopic tumor cell embolism. Tumor emboli do not occlude affected vessels but induce both local activation of coagulation and fibrocellular intimal proliferation, which lead into stenosis or occlusion. Hemodynamically, an increase in vascular resistance results in pulmonary hypertension. In three patients, metastatic carcinoma was unknown before death, and the condition was diagnosed as pulmonary hypertension of unknown origin. Thus, pulmonary tumor thrombotic microangiopathy should be considered in the differential diagnosis of primary pulmonary hypertension, particularly in patients with well‐known carcinoma who develop acute or subacute cor pulmonale.