title: Novel management of anticoagulant resistant thrombotic hypodysfibrinogenaemia.
abstract: 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.
authors.affiliation: Department of Thrombosis & Haemophilia, Guy's & St Thomas' NHS Foundation Trust, London SE1 7EH.
authors.forename: C
authors.initials: C
authors.lastname: Rea
authors.forename: B J
authors.initials: BJ
authors.lastname: Hunt
mesh_terms: D000328:Adult; D000347:Afibrinogenemia / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000628:therapy*; D004351:Drug Resistance; D006801:Humans; D008297:Male; D013927:Thrombosis / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000628:therapy*; D055815:Young Adult