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factor XIII deficiency
Genetics:
- autosomal recessive
- deficiency of defect in factor XIII or its subunits
Clinical manifestations:
1) severe bleeding problems
2) severe scarring with superficial wounds
Laboratory:
1) aPTT is normal
2) PT is normal
3) bleeding time is normal
4) plasma clot is abnormally soluble in 5 molar urea
5) factor XIII activity in plasma
6) factor XIII antigen in plasma
7) factor XIII coagulum dissolution in plasma
* also see ARUP consult [1]
Management: prophylactic factor XIII every 3-4 weeks.
Related
coagulation factor XIII; fibrin-stabilizing factor
Specific
F13A deficiency
F13B deficiency
General
genetic disease of the blood/bone marrow
References
- ARUP consult:
Factor XIII Deficiency Testing
https://arupconsult.com/ati/factor-xiii-deficiency-testing