Which patient groups have the highest prevalence of factor XIII (FXIII) deficiency?

Updated: Aug 01, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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No racial predilection exists for FXIII deficiency. FXIII deficiency has been reported widely. The restriction of certain polymorphisms to specific populations should be expected.

Since it is an autosomal disorder, homozygous FXIII deficiency occurs in either sex. Acquired inhibitors to FXIII can present in either males or females.

Physiologically, reduced levels of FXIII are found in healthy newborns, with a gradual rise in levels into the reference range. Premature infants have lower values than full-term neonates. FXIII levels drop in the latter half of a normal pregnancy.

Neonates with severe FXIII deficiency may present with bleeding from the umbilical cord. Easy bruising and delayed and recurrent bleeding after trauma begin in childhood. Oral bleeding can begin with teething and cuts or abrasions to the lips, tongue, and frenulum. Bleeding remains a problem throughout life and requires replacement therapy. FXIII deficiency acquired as a result of autoantibodies has been reported in the older population, as has acquired hemophilia A. Both drug-induced autoantibodies and alloantibodies have been reported in severely deficient patients who have been receiving replacement therapy.

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