What are the signs and symptoms of factor XIII (FXIII) deficiency?

Updated: Mar 09, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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The following symptoms should trigger an evaluation for FXIII deficiency:

  • Spontaneous miscarriages early in pregnancy

  • Bleeding from the umbilical cord 1-19 days after birth

  • Easy bruising and soft tissue bleeding, particularly in association with trauma, as the infant starts to ambulate; bleeding following trauma may be immediate, delayed, and/or recurrent

  • CNS hemorrhage is common, recurs in approximately 30% of patients, and may be the initial manifestation in patients with severe FXIII deficiency

  • CNS bleeding may be preceded by head trauma in children, while adults may develop a CNS bleed in the absence of obvious trauma

  • Symptoms typical of any CNS event may be present (eg, headaches, seizures, vomiting, focal neurologic defects); symptoms may be acute at onset or may be superimposed on residual findings of a past bleed

  • Menorrhagia and intra-abdominal bleeding during menses

  • Bleeding into joints

  • Poor wound healing, although described, is less common

  • Autoantibodies to FXIII are an acquired cause of a bleeding diathesis; these may be triggered by isoniazid, so a detailed drug history is essential

  • Therapeutic plasma exchange can result in acquired FXIII deficiency; it may be underdiagnosed, as routine coagulation tests remain unaltered, but significant bleeding may ensue, especially in patients who have undergone recent surgery, such as a kidney transplant. [4]

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