Rare Coagulation Disorders Fibrinogen deficiency

The hypo- and dysfibrinogenemias comprise a collection of disorders that are usually dominantly inherited and associated with both bleeding and venous thrombotic manifestations. Women are at risk of recurrent miscarriage, and both antenatal and postnatal hemorrhage. In hypofibrinogenemia, both antigenic and functional fibrinogen levels are reduced. The diagnosis of dysfibrinogenemia is made by demonstrating a prolonged TT with a normal antigenic fibrinogen level.

Prophylaxis with fibrinogen concentrates improves pregnancy outcome and prevents antepartum and postpartum hemorrhage in women with hypo- and dysfibrinogenemia. Cryoprecipitate is a good source of fibrinogen but should not usually be used, as it is not virally inactivated. Its use may be considered in an emergency situation if no other alternatives are available. The half-life of infused fibrinogen is 3-5 days, and treatment is unlikely to be needed more often than on alternate days. Levels above 1.5 g/l are required toward the end of pregnancy and at the time of delivery75.

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