FACTOR XIII DEFICIENCY
- Factor XIII deficiency – 1 in 2,000,000 (higher in southeastern Iran)
- Males and Females equally affected
- Often a Family History, screening is advised.
- Excessive bleeding with trauma or invasive procedures such as circumcision or tooth extraction
- Menorrhagia and/or postpartum bleeding
- Muscle and joint bleeding
- Mucosal tract bleeding
- Conservative "watch and wait"
- If bleeding: recombinant factor XIII A subunit of factor XIII concentrate
- Consider prophylaxis if levels <5%.
.DARE TO KNOW.
- Factor XIII enzyme is a heterotetramer consisting of two catalytic A subunits and two B subunits, most deficiencies are due to defects in the A subunit
- PT and aPTT normal
- clot solubility in 5M urea may be enhanced