What is the mortality and morbidity associated with factor XIII (FXIII) deficiency?

Updated: Apr 02, 2018
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Answer

Umbilical bleeding starting in the first few days after birth, recurrent intracranial bleeding, and recurrent early miscarriages are hallmarks of FXIII deficiency.

Approximately 30% of central nervous system (CNS) bleeding is recurrent, and approximately 50% of CNS bleeding may be fatal, but the severity of bleeding varies from family to family. Posttraumatic bleeding may be immediate, delayed, or recurrent. Traumatic joint bleeding may develop. Poor wound healing has been described, although this is not a universal finding.

Cryoprecipitate and fresh frozen plasma (FFP) provide a source of FXIII for most patients. All plasma-derived products carry risks of transmitting hepatitis, HIV, parvovirus B19, transfusion-transmitted virus (TTV), and prion-induced (new variant Creutzfeldt-Jacob disease [nvCJD]) illnesses (see Complications and the Medscape article Hemophilia A for more information). A recombinant factor XIII (rFXIII) subunit A concentrate is available for use in congenital FXIII A-subunit deficiency. [71]

Development of FXIII inhibitors (alloantibodies or autoantibodies) is associated with significant morbidity and mortality.

Pregnant women with FXIII deficiency have a significant risk of miscarriage, placental abruption, and postpartum hemorrhage without prophylaxis. [72]


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