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血友病患者 FV 和 VIII 缺乏症并发肺栓塞:病例报告。

Pulmonary embolism in a hemophiliac patient with factor V and VIII deficiency: a case report.

机构信息

Clinical Fellow in Obstetrics Anaesthetic Department, Leicester Royal Infirmary, Leicester, UK.

Consultant anaesthetists, Leicester Royal Infirmary, Leicester, UK.

出版信息

J Med Case Rep. 2024 Oct 19;18(1):481. doi: 10.1186/s13256-024-04831-6.

DOI:10.1186/s13256-024-04831-6
PMID:39425180
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11490163/
Abstract

BACKGROUND

This is an interesting and unexpected thromboembolic event in a hemophiliac patient.

CASE PRESENTATION

This is a case of a 33-year-old primigravidarum of Indian Asian origin with Factor V and Factor VIII deficiency who developed a case of pulmonary embolism during the course of her pregnancy after presenting to the emergency department in Leicester, United Kingdom, with hemoptysis, tachycardia, and tachypnea. Patient was subsequently diagnosed with pulmonary embolism after a computed tomography pulmonary angiogram and was treated with therapeutic daltaparin, a low-molecular-weight heparin.

CONCLUSION

In conclusion, a thromboembolic event is not an improbable event in a hemophiliac patient and should be considered despite a low Well's score.

摘要

背景

这是一位血友病患者中出现的有趣且意外的血栓栓塞事件。

病例介绍

这是一位来自印度裔的 33 岁初产妇,患有因子 V 和因子 VIII 缺乏症,在英国莱斯特的急诊室因咯血、心动过速和呼吸急促就诊后,在怀孕期间发生了肺栓塞。患者随后通过计算机断层扫描肺动脉造影被诊断为肺栓塞,并接受了达肝素(一种低分子量肝素)的治疗。

结论

总之,即使 Wells 评分较低,也应考虑到血栓栓塞事件在血友病患者中并非不可能发生。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e8f/11490163/5d9a0b3904ba/13256_2024_4831_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e8f/11490163/02358cb1299e/13256_2024_4831_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e8f/11490163/5d9a0b3904ba/13256_2024_4831_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e8f/11490163/02358cb1299e/13256_2024_4831_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e8f/11490163/5d9a0b3904ba/13256_2024_4831_Fig2_HTML.jpg

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本文引用的文献

1
Pulmonary Embolism in Acquired Hemophilia A: A Rare Complication With Factor VIII Inhibitor Bypassing Activity Therapy.获得性血友病A中的肺栓塞:一种使用凝血因子VIII抑制物旁路活性治疗的罕见并发症。
Cureus. 2020 Jul 12;12(7):e9152. doi: 10.7759/cureus.9152.
2
Post splenectomy fatal pulmonary embolism in a patient with moderate hemophilia a.中度甲型血友病患者脾切除术后致命性肺栓塞
Iran J Med Sci. 2011 Jun;36(2):136-40.
3
Combined Factor V and Factor VIII Deficiency.联合因子V和因子VIII缺乏症。
Semin Thromb Hemost. 2009 Jun;35(4):390-9. doi: 10.1055/s-0029-1225761. Epub 2009 Jul 13.
4
Combined FV and FVIII deficiency.联合因子V和因子VIII缺乏症。
Haemophilia. 2008 Nov;14(6):1201-8. doi: 10.1111/j.1365-2516.2008.01845.x.
5
Prediction of pulmonary embolism in the emergency department: the revised Geneva score.急诊科肺栓塞的预测:修订的日内瓦评分
Ann Intern Med. 2006 Feb 7;144(3):165-71. doi: 10.7326/0003-4819-144-3-200602070-00004.
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Evaluation of D-dimer in the diagnosis of suspected deep-vein thrombosis.D-二聚体在疑似深静脉血栓形成诊断中的评估
N Engl J Med. 2003 Sep 25;349(13):1227-35. doi: 10.1056/NEJMoa023153.
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Venous thromboembolism: pathophysiology, clinical features, and prevention.静脉血栓栓塞症:病理生理学、临床特征及预防
BMJ. 2002 Oct 19;325(7369):887-90. doi: 10.1136/bmj.325.7369.887.