Congenital Blood Coagulation Factor XIII Deficiency and Successful Deliveries: A Review of the Literature

Abstract

Congenital deficiency of blood coagulation factor XIII is an uncommon, inherited disorder characterized by hemorrhagic diathesis, habitual abortions and defective wound healing. We analyzed 8 reported successful pregnancies in women with a congenital deficiency of A-subunit of factor XIII (XIIIA), in which the plasma level of maternal factor XIIIA and/or the precise replacement therapies are described. Because decidual bleeding usually begins from 5 to 6 weeks' gestation and, without replacement therapy, spontaneous abortion always occurs, we herein offer the following prenatal and peripartum management guidelines and observations: i) the level of plasma A-subunit of factor XIII antigen (XIIIA-Ag) or factor XIII activity (XIII-act) must be at least 2%-3%, and, if possible, higher than 10% to prevent decidual bleeding and miscarriage during the pregnancy; ii) factor XIIIA concentrate is better than fresh frozen plasma or cryoprecipitate for replacement therapy; iii) the administration of 250 international units (IU) every 7 days is sufficient to maintain the level of plasma XIIIA-Ag or XIII-act more than 10% in the early period of gestation (through 22 weeks' gestation); however, 500 IU every 7 days is indicated in the later period (from 23 weeks' gestation) to maintain that level; iv) during labor, the desired level of plasma XIIIA-Ag or XIII-act should be higher than 20%, and, if possible, higher than 30% in order to make ready for any risk of severe obstetrical hemorrhagic complications; thus a booster dose of 1000 IU is indicated before labor; v) no replacement therapy is necessary in the puerperium because it is usually uneventful without it.

An unusual clinical presentation of factor XIII deficiency and issues relating to the monitoring of factor XIII replacement therapy

“…In patients with known congenital FXIII deficiency, replacement therapy throughout pregnancy results in successful outcomes [15][16][17]. Different regimens of prophylaxis have been used in different countries [15,18], but there is no validated recommendation regarding the optimal dose of FXIII during pregnancy. In this patient, FXIII 20 IU/kg given every 3 weeks prevented bleeding and miscarriage, but it is important to note that a shortened and dilated cervix was detected early in the pregnancy.…”

Karyotype-Phenotype Correlation in Partial Trisomies of the Short Arm of Chromosome 6: A Family Case Report and Review of the Literature

“…Classically considered as haemorrhagic disorder, it can result in a lifelong bleeding tendency [Komáromi et al, 2011] and defective wound healing and tissue repair [Inbal and Dardik, 2006]. It was recently found to be associated with chronic venous leg ulcers [Zamboni and Gemmati, 2007] and recurrent abortion [Asahina et al, 2007]. High factor XIII circulating levels can be detected among thrombophilic patients [Muszbek et al, 2010].…”

Novel aspects of factor XIII deficiency

“…Limited data on prophylaxis during pregnancy are available; a plasma FXIII level above 10% seems to be sufficient for successful pregnancy. Asahina et al [9] have reported that 250 IU per week were sufficient to maintain this level in the early period of gestation, and 500 IU per week is recommended after the 23rd week of gestation. The same authors propose that plasma FXIII level should be higher than 30% during labor and a booster dose of 1000 IU is recommended before labor to prevent severe obstetrical hemorrhagic complication.…”