factor XIII deficiency

Genetics

• autosomal recessive
• deficiency of defect in factor XIII or its subunits

Clinical manifestations

• severe bleeding problems
• severe scarring with superficial wounds

Laboratory

• aPTT is normal
• PT is normal
• bleeding time is normal
• plasma clot is abnormally soluble in 5 molar urea
• factor XIII activity in plasma
• factor XIII antigen in plasma
• factor XIII coagulum dissolution in plasma

* also see ARUP consult

Management

prophylactic factor XIII every 3-4 weeks

More general terms

• genetic disease of the blood/bone marrow

More specific terms

• F13A deficiency
• F13B deficiency

Additional terms

• coagulation factor XIII; fibrin-stabilizing factor