Novel management of anticoagulant resistant thrombotic hypodysfibrinogenaemia

Individuals with hypodysfibrinogenaemia and recurrent thrombosis are rarely encountered and there is no consensus regarding long-term management. Guidelines available suggest that oral anticoagulants should be sufficient treatment, but when this fails to control thrombotic disease no further management strategies are reported. A novel approach to treatment has been developed and used for two individuals who experienced life-threatening thrombosis despite anticoagulation adherent to current guidelines. These two affected individuals consented to receive infusions of exogenous fibrinogen concentrate thrice weekly in addition to continuing warfarin (target INR 3-4). Both have been thrombosis free 36 and 18 months after starting this ongoing regime. This study suggests regular transfusions of fibrinogen concentrate may be a suitable treatment for anticoagulant resistant thrombotic hypodysfibrinogenaemia, but further research is required.