Understanding FXIII and FVII Deficiencies

Women with congenital FXIII deficiency need to be carefully monitored during pregnancy

WFH 2016 (Orlando, July 24-28): Rare bleeding disorders were discussed at one of the educational sessions at the World Federation of Hemophilia congress. Dr. Diane Nugent from CIBD presented an overview of congenital FXIII deficiency with a focus on issues of FXIII deficiency during pregnancy. Congenital FXIII deficiency is one of the rarest of the factor deficiencies and occurs in about 1 per 3 to 5 million people, affecting both men and women equally. Some of the symptoms of this rare bleeding disorder include bruising, nose and mouth bleeds, muscle bleeds, surgical bleeding, poor wound healing, and spontaneous bleeds in the brain. Miscarriages and heavy menstrual bleeding also frequently occur in women. It is well known that FXIII levels in unaffected (normal) pregnant women steadily drop with each trimester. Therefore, in pregnant women with congenital FXIII deficiency, it is important to check levels and give replacement treatment as needed since the factors are consumed more rapidly.