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真性红细胞增多症:外周血管表现

POLYCYTHEMIA VERA: PERIPHERAL VASCULAR MANIFESTATIONS.

作者信息

MOORE W S, BLAISDELL F W, HALL A D

出版信息

Calif Med. 1964 Feb;100(2):92-6.

Abstract

The presenting manifestations of polycythemia vera are often complications involving the vascular system. These include myocardial infarction, cerebro-vascular accidents and ischemic changes in the extremities.The concept of increased atherogenesis in cases of polycythemia vera has been questioned. A possible mechanism by which small, otherwise subclinical atheromatous plaques produce ischemic symptoms in patients with polycythemia vera is discussed. The blood in polycythemic patients has been shown to have an increased viscosity resulting in a prolonged circulation time. If a small atheromatous plaque is present in association with increased blood viscosity, this combination may well produce ischemic symptoms. This explains why treatment of polycythemia vera, with restoration of blood to normal viscosity, often reverses the patient's ischemic symptoms. Two cases of polycythemia vera here reported, in which the presenting manifestations were gangrenous extremities, emphasize the need for prompt diagnosis and treatment of polycythemia vera. In the first case, early recognition and treatment of polycythemia vera successfully reversed the ischemic changes in the extremities, while failure of early recognition and treatment in the second case resulted in two major amputations.

摘要

真性红细胞增多症的临床表现通常是涉及血管系统的并发症。这些并发症包括心肌梗死、脑血管意外以及四肢的缺血性改变。真性红细胞增多症患者动脉粥样硬化形成增加这一概念受到了质疑。本文讨论了一种可能的机制,即原本较小的、临床上未表现出症状的动脉粥样硬化斑块如何在真性红细胞增多症患者中引发缺血症状。研究表明,真性红细胞增多症患者的血液粘度增加,导致循环时间延长。如果存在一个小的动脉粥样硬化斑块,同时血液粘度又增加,那么这种组合很可能会引发缺血症状。这就解释了为什么对真性红细胞增多症进行治疗,使血液粘度恢复正常后,常常能逆转患者的缺血症状。本文报告了两例真性红细胞增多症病例,其临床表现均为四肢坏疽,强调了对真性红细胞增多症进行及时诊断和治疗的必要性。在第一例中,对真性红细胞增多症的早期识别和治疗成功逆转了四肢的缺血性改变,而在第二例中,由于早期识别和治疗失败,导致患者进行了两次大截肢手术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/13b9/1515149/a380e66a8b96/califmed00080-0010-a.jpg

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