Umbilical stump bleeding (>90%), intracranial
bleeding (>25%), superficial bleeding/subcutaneous hematomas (>50%),
hemarthrosis (25%), normal prothrombin time, partial thromboplastin time,
and platelet count. Treatment usually entails fresh-frozen plasma 5 ml/kg at
monthly intervals as prophylaxis. Treatment with cryoprecipitate is
effective. Virally inactivated FXIII concentrates exist but are reportedly
not commercially available everywhere.