获得性因子V抑制剂在儿童静脉血栓形成中的应用。

IF 3.4 3区医学 Q2 HEMATOLOGY

Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-01-01 DOI:10.1016/j.rpth.2024.102646

Sweta Gupta , Matthew W. Bunce , Emily A. Cid , Rodney M. Camire , Amy D. Shapiro

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引用次数: 0

摘要

背景：获得性因子(F)V与抑制剂（AFVwI）的发展是罕见的，主要导致出血并发症，尽管在成人中有零星的血栓病例报道。关键临床问题：您如何诊断和处理与AFVwI并发发现相关的急性深静脉血栓患儿？临床方法：一名患有克罗恩病和May-Thurner解剖的13岁女性左下肢广泛深静脉血栓形成，并发AFVwI，在评估凝血酶原时间延长和低FV活性时发现。抗凝治疗开始使用低分子肝素，随后直接口服抗凝剂利伐沙班，无任何并发症。5个月后，FV活性恢复正常，AFVwI未检出。结论：我们的病例强调了第一例小儿血栓形成与罕见的发现AFVwI，成功地管理与抗凝治疗完全解决。

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Acquired factor V inhibitor in a case of pediatric venous thrombosis

Background

The development of acquired factor (F)V with inhibitor (AFVwI) is rare, resulting mainly in bleeding complications, although sporadic cases of thrombosis in adults have been reported.

Key Clinical Question

How do you diagnose and manage a pediatric case of acute deep venous thrombosis associated with the concurrent finding of AFVwI?

Clinical Approach

A 13-year-old female with Crohn's Disease and May–Thurner anatomy developed extensive deep venous thrombosis of the left lower extremity, complicated by the finding of AFVwI, discovered during the evaluation of a prolonged prothrombin time and a low FV activity. Anticoagulation was initiated with low-molecular-weight heparin followed by a direct oral anticoagulant, rivaroxaban, without any complications. AFVwI was undetectable after 5 months with normalization of FV activity.

Conclusion

Our case highlights the first pediatric case of thrombosis with a rare finding of AFVwI, successfully managed with anticoagulation therapy with complete resolution.

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