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Published on: July 4, 2021
Multivessel thromboembolism associated with dysfunction of protein s
Eun Jae Ok1, Hye Won Kim, Sang Dong Kim
1Department of Rehabilitation Medicine, College of Medicine, The Catholic University of Korea, Suwon 442-723, Korea.
Isolated protein S deficiency can cause severe arterial thromboembolic events, even in young adults. Early diagnosis and evaluation are crucial for managing these life-threatening conditions.
Area of Science:
- Hematology
- Vascular Medicine
- Genetics
Background:
- Protein S is a key anticoagulant vitamin K-dependent protein.
- Protein S deficiency is linked to an increased risk of venous and arterial thrombosis.
- Arterial thromboembolic events are less commonly associated with protein S deficiency compared to venous events.
Purpose of the Study:
- To report a case of arterial occlusion due to isolated protein S deficiency.
- To highlight the importance of considering protein S deficiency in young adults with arterial thromboembolic events.
Main Methods:
- Case report of a 39-year-old woman with bilateral lower leg arterial occlusion.
- Diagnostic workup included imaging for thromboembolism and protein S level assessment.
- Surgical interventions: thrombectomy, angioplasty, and amputation.
- Medical management with systemic anticoagulation.
Main Results:
- The patient presented with arterial occlusion in both lower legs, requiring surgical intervention and amputation.
- Further evaluation revealed a large right atrial thrombus and asymptomatic pulmonary thromboembolism.
- Isolated protein S deficiency was diagnosed as the underlying cause.
- Successful treatment with right atrial thrombectomy and anticoagulation.
Conclusions:
- Isolated protein S deficiency should be considered in the differential diagnosis of arterial thromboembolic events, particularly in young adults.
- Comprehensive evaluation for coagulation disorders is essential in patients presenting with unexplained arterial thrombosis.
- Prompt diagnosis and management of protein S deficiency can prevent recurrent thromboembolic events.
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