Bounaix Laura, Schroeder Verena, Fontana Pierre, Casini Alessandro
Division of Angiology and Haemostasis, Geneva University Hospitals and Faculty of Medicine, Geneva, Switzerland.
Experimental Haemostasis Group, Department for BioMedical Research (DBMR), University of Bern, Bern, Switzerland.
Res Pract Thromb Haemost. 2024 Mar 15;8(2):102371. doi: 10.1016/j.rpth.2024.102371. eCollection 2024 Feb.
Thrombotic events in congenital factor (F)XIII deficiency are extremely rare. To our knowledge, we describe the first case of severe congenital FXIII deficiency associated with recurrent venous thrombotic events.
How to deal with anticoagulation treatment in patients with severe FXIII deficiency?
The patient was treated with rivaroxaban and plasma-derived FXIII substitution therapy as prophylaxis without bleeding complications. We aimed at FXIII trough levels of 50% during the loading doses of rivaroxaban, then 30% during the maintenance dose of rivaroxaban, and finally 20% during the long-term use of prophylactic dose of rivaroxaban.
Treatment of thrombotic events with rivaroxaban in patients with severe bleeding disorders seems to be safe, requiring an adaptation of the intensity of the replacement therapy.
先天性因子（F）XIII缺乏症中的血栓形成事件极为罕见。据我们所知，我们描述了首例与复发性静脉血栓形成事件相关的严重先天性FXIII缺乏症病例。
如何处理严重FXIII缺乏症患者的抗凝治疗？
该患者接受利伐沙班和血浆源性FXIII替代疗法作为预防措施，未出现出血并发症。我们的目标是在利伐沙班负荷剂量期间使FXIII谷浓度达到50%，然后在利伐沙班维持剂量期间达到30%，最后在长期使用利伐沙班预防剂量期间达到20%。
在严重出血性疾病患者中使用利伐沙班治疗血栓形成事件似乎是安全的，需要调整替代治疗的强度。
