Novel management of anticoagulant resistant thrombotic hypodysfibrinogenaemia.

Friday, 20 January, 2012

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.

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Published: 2012 Nov;130(5):785-7. doi: 10.1016/j.thromres.2011.12.027. Epub 2012 Jan 20.