Dysfibrinogenaemia

4 PT and APTT. Variable abnormalities of thrombin time and reptilase time; fibrinogen-dependent platelet function may be defective. Confirm diagnosis by demonstrating normal chemical/immunological fibrinogen concentrations with reduced functional properties.

Treatment

Fibrinogen has a long t,4 (3—5d), severe deficiency managed by repeated (twice weekly) prophylactic injections with fibrinogen concentrates, FFP or cryoprecipitate. Fibrinogen levels should be raised to 0.5-1.0g/L to achieve haemostasis.

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