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年轻患者合并静脉和动脉血栓形成,提示潜在骨髓增殖性疾病:病例报告。

Combined venous and arterial thrombosis revealing underlying myeloproliferative disorder in a young patient: a case report.

机构信息

Cardiology department, Hospital University Center Ibn Rochd, Casablanca, Morocco.

出版信息

J Med Case Rep. 2021 Feb 16;15(1):76. doi: 10.1186/s13256-020-02593-5.

Abstract

BACKGROUND

Myeloproliferative neoplasms (MPNs) such as polycythemia Vera (PV) and Essential Thrombocythemia (ET) can be associated with a high risk of both venous and arterial thrombosis. However, the co-existence between these two complications is very rare and has never been described before, especially in young adults with no known history of MPNs.

CASE PRESENTATION

We report the case of a 39 year-old Caucasian Moroccan male patient without cardiovascular risk factors (CVRF), who presented with acute chest pain. He also suffered from a severe headache since 2 weeks. Electrocardiogram (ECG) showed ST segment elevation myocardial infarction in the posterolateral leads. Cerebral Computed Tomography (CT) scan revealed subarachnoid hemorrhage (SAH), and cerebral Magnetic Resonance Angiography (MRA) found a Superior Sagittal Sinus Thrombosis (SSST). Routine blood tests showed raised hemoglobin and hematocrit in addition to leukocytosis and thrombocythemia. His coronary angiography revealed a thrombus in the ostial left circumflex artery (LCX). Further testing revealed positive Janus kinase 2 (JAK2) V617F mutation and low erythropoietin level, confirming the diagnosis of PV according to the 2008 World Health Organization (WHO) criteria. Antithrombotic and anti-ischemic treatments, in addition to myelosuppressive therapy with hydroxyurea, were initiated with a good clinical and biological evolution.

CONCLUSION

This case shows that MPNs are an important cause of thrombosis, especially in young patients with no other risk factors. Early diagnosis and appropriate management are fundamental before the occurrence of life-threatening complications that can sometimes present in unusual forms associating arterial and venous thrombotic events.

摘要

背景

骨髓增殖性肿瘤(MPNs),如真性红细胞增多症(PV)和原发性血小板增多症(ET),可伴发静脉和动脉血栓形成的高风险。然而,这两种并发症同时存在非常罕见,以前从未有过描述,特别是在没有已知 MPNs 病史的年轻成年人中。

病例介绍

我们报告了一例 39 岁的高加索裔摩洛哥男性患者,无心血管危险因素(CVRF),因急性胸痛就诊。他还在 2 周前出现严重头痛。心电图(ECG)显示后外侧导联 ST 段抬高型心肌梗死。脑计算机断层扫描(CT)显示蛛网膜下腔出血(SAH),脑磁共振血管造影(MRA)发现上矢状窦血栓形成(SSST)。常规血液检查显示血红蛋白和血细胞比容升高,白细胞增多和血小板增多。冠状动脉造影显示左回旋支(LCX)开口处血栓形成。进一步检查发现阳性 Janus 激酶 2(JAK2)V617F 突变和低促红细胞生成素水平,根据 2008 年世界卫生组织(WHO)标准确诊为 PV。启动了抗血栓和抗缺血治疗,以及羟基脲的骨髓抑制治疗,患者的临床和生物学状况均有良好改善。

结论

该病例表明 MPNs 是血栓形成的重要原因,尤其是在无其他危险因素的年轻患者中。在发生危及生命的并发症之前,早期诊断和适当的管理非常重要,有时这些并发症会以不常见的形式同时出现,合并动脉和静脉血栓形成事件。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/174d/7885597/02cbeb40a58d/13256_2020_2593_Fig1_HTML.jpg

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