E. Zanon

Background: FXIII deficiency is a very rare coagulation disorder Bleeds are usually muscular or mucocutaneous, but bleeding from umbilical cord at birth and intracranial hemorrhages (ICHs) can occur in cases of severe disease Plasma derived concentrates (Fibrogammin ®) and RFXIII concentrates (Novothirteen ®) can be used on demand to control the bleeding. The best treatment in patients with severe disease is the use of plasma derived or recombinant FXIII concentrates on prophylaxis at the dose of 10 26 IU/kg every 4 6 weeks and 35 IU/kg every 4 weeks, respectively Few data on the use of rFXIII in the real world scenario are available.

Material: and methods recently we enrolled all patients presenting FXIII deficiency treated with catridecacog at ten Italian Hemophilia Centers PK-profiles were evaluated and clinical data and outcomes were collected and analyzed...

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