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Scientific article
Case report
Open access
English

Management of anticoagulation and factor XIII replacement in a patient with severe factor XIII deficiency and recurrent venous thromboembolic disease: case report and review of literature

Published inResearch and practice in thrombosis and haemostasis, vol. 8, no. 2, 102371
Publication date2024-02
First online date2024-03-15
Abstract

Background: 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.

Key clinician question: How to deal with anticoagulation treatment in patients with severe FXIII deficiency?

Clinical approach: 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.

Conclusion: 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.

eng
Keywords
  • Anticoagulation
  • Congenital deficiency
  • Factor XIII
  • Thrombosis
Citation (ISO format)
BOUNAIX, Laura et al. Management of anticoagulation and factor XIII replacement in a patient with severe factor XIII deficiency and recurrent venous thromboembolic disease: case report and review of literature. In: Research and practice in thrombosis and haemostasis, 2024, vol. 8, n° 2, p. 102371. doi: 10.1016/j.rpth.2024.102371
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Identifiers
ISSN of the journal2475-0379
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