FACTOR XIII (FXIII)

Autosomal recessive inheritance.
Umbilical cord bleeding is common in FXIII deficiency.
Some patients sustain a spontaneous intracranial hemorrhage, a brain bleed, which is the leading cause of mortality. Other symptoms of FXIII deficiency include bruising, nose and mouth bleeds, muscle bleeds and delayed bleeding after surgery.
Women can experience menorrhagia, long, heavy menstrual periods, and repeat miscarriages.
Men with FXIII deficiency may show signs of infertility.
Acquired FXIII deficiency can be observed in presence of autoantibodies.