Nand S, Orfei E
Department of Medicine, Loyola University of Chicago, Stritch School of Medicine, Maywood, Illinois 60153.
Am J Hematol. 1994 Nov;47(3):242-4. doi: 10.1002/ajh.2830470320.
Thromboembolic events occur in about 27% of the patients with polycythemia vera and account for 31% of the deaths. These include cerebrovascular accidents, myocardial infarction, peripheral vascular occlusions, pulmonary infarctions, and venous thrombosis. We report two cases with polycythemia vera who presented with pulmonary hypertension in the absence of previous thromboembolic complications of any kind. One patient died suddenly, with evidence of extensive bilateral thrombosis of prelobular pulmonary arteries at autopsy. In the second patient, local thrombosis in the pulmonary vasculature or recurrent silent pulmonary emboli appear to be responsible for the development of pulmonary hypertension. After institution of anticoagulant therapy, he is able to maintain his functional status. The purpose of this report is to alert clinicians to the development of this insidious, but potentially fatal complication in patients with polycythemia vera.
血栓栓塞事件发生在约27%的真性红细胞增多症患者中，占死亡人数的31%。这些事件包括脑血管意外、心肌梗死、外周血管闭塞、肺梗死和静脉血栓形成。我们报告两例真性红细胞增多症患者，他们在没有任何既往血栓栓塞并发症的情况下出现了肺动脉高压。一名患者突然死亡，尸检显示小叶前肺动脉广泛双侧血栓形成。在第二名患者中，肺血管局部血栓形成或反复无症状肺栓塞似乎是肺动脉高压发展的原因。在开始抗凝治疗后，他能够维持其功能状态。本报告的目的是提醒临床医生注意真性红细胞增多症患者中这种隐匿但可能致命的并发症的发生。
