Congenital blood coagulation factor XIII deficiency and successful deliveries: a review of the literature.
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.
Asahina
Toshihiko
T
Department of Obstetrics and Gynecology, Hamamatsu University School of Medicine, Hamamatsu, Shizuoka, Japan. mkt51440@po3.across.or.jp
Kobayashi
Takao
T
Takeuchi
Kinya
K
Kanayama
Naohiro
N
D000022:Abortion, Spontaneous / Q000209:etiology / Q000517:prevention & control*; D005177:Factor XIII Deficiency / Q000151:congenital* / Q000188:drug therapy; D026122:Factor XIIIa / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D017410:Practice Guidelines as Topic; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000188:drug therapy*; D014592:Uterine Hemorrhage / Q000209:etiology
D026122:Factor XIIIa