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熄灯、开灯:真性红细胞增多症中的一过性黑矇

Lights Off, Lights On: Amaurosis Fugax in Polycythemia.

作者信息

Tan Shao Sze, Samsudin Amir, Thavaratnam Lakana Kumar, Nurul-Ain Masnon

机构信息

Ophthalmology, Hospital Seremban, Seremban, MYS.

Ophthalmology, University of Malaya, Kuala Lumpur, MYS.

出版信息

Cureus. 2022 Jun 8;14(6):e25752. doi: 10.7759/cureus.25752. eCollection 2022 Jun.

Abstract

There are many causes of amaurosis fugax, including polycythemia. Polycythemia is associated with elevated hematocrit levels and hyperviscosity, which can lead to ocular manifestations. We report a polycythemia patient with amaurosis fugax, who had resolution of ocular symptoms following venesection. A 29-year-old gentleman presented with a six-month history of episodic bilateral transient loss of vision (amaurosis fugax), followed by slow recovery back to normal after 15-20 minutes. The symptoms worsened with fatigue. He also had an unsteady gait for the preceding one year. Ocular examination was unremarkable. His visual acuity was 20/20 OU. Neurological examination revealed gait ataxia and dysdiadochokinesia. Computed tomography (CT) angiogram showed an old cerebellar infarct. Blood investigations showed persistent elevated hemoglobin and hematocrit with positive JAK-2 V617F mutation. Infective and connective tissue workups were all negative. A diagnosis of polycythemia was made by the haematology team. In addition to oral aspirin given by the neurology team, he underwent venesection with improvement in ocular symptoms following each episode of venesection. The frequency of amaurosis fugax reduced from 2-3 episodes a week to once a month, then resolved completely after five venesections. Systemically, his cerebellar symptoms also resolved and there were no neurological deficits. Polycythemia is a rare disease that can cause amaurosis fugax and thrombotic events in young patients. Better knowledge and accurate diagnosis are important, as early treatment may improve the symptoms and long-term morbidity.

摘要

一过性黑矇有多种病因,包括红细胞增多症。红细胞增多症与血细胞比容水平升高及血液高黏滞度有关,可导致眼部表现。我们报告一例红细胞增多症合并一过性黑矇的患者,其眼部症状在放血治疗后得到缓解。一名29岁男性,有6个月发作性双侧短暂视力丧失(一过性黑矇)病史,发作后15 - 20分钟缓慢恢复至正常。症状在疲劳时加重。他在之前一年还出现步态不稳。眼部检查无异常。双眼视力均为20/20。神经系统检查发现步态共济失调和轮替运动障碍。计算机断层扫描(CT)血管造影显示陈旧性小脑梗死。血液检查显示血红蛋白和血细胞比容持续升高,JAK - 2 V617F突变阳性。感染性和结缔组织检查均为阴性。血液科团队做出红细胞增多症的诊断。除了神经科团队给予的口服阿司匹林外,他接受了放血治疗,每次放血后眼部症状均有改善。一过性黑矇的发作频率从每周2 - 3次降至每月1次,在5次放血后完全缓解。全身方面,他的小脑症状也得到缓解,且无神经功能缺损。红细胞增多症是一种罕见疾病,可在年轻患者中引起一过性黑矇和血栓形成事件。更好的认识和准确的诊断很重要,因为早期治疗可能改善症状和长期发病率。

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