ISTH Academy

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.

• Thrombotic events in congenital factor XIII (FXIII) deficiency are extremely rare- Anticoagulation in patient with a rare bleeding disorder is a challenging clinical situation. • We adapted the prophylacitc FXIII replacement according to the intensity of anticoagulation. • Rivaroxaban seems safe for treating and preventing venous thromboembolism in patients with FXIII deficiency.