Kumar M, Mehta P
Division of Pediatric Hematology/Oncology, University of Florida, Gainesville 32610.
Am J Hematol. 1994 Jul;46(3):241-4. doi: 10.1002/ajh.2830460315.
Pregnancy in women with congenital coagulation factor deficiencies has been associated with adverse fetal outcomes. Recurrent spontaneous abortions, placental abruptions, and premature births are reported, the exact reasons for which are not clear, and management of such patients continues to be a challenge. We reviewed the outcome of four pregnancies in a patient with factor X deficiency, and looked at the effect of factor replacement therapy on pregnancy. Her first two pregnancies resulted in the birth of extremely premature babies at 21 and 25 weeks of gestation, both of which died in the neonatal period. The patient was treated with fresh frozen plasma for acute bleeding episodes during these pregnancies. In addition during her second conception she was given factor IX complex [Konyne] prophylactically, but only in the latter half of her pregnancy. During her next two pregnancies she was treated early on during pregnancy, with prophylactic replacement of factor X. She delivered healthy babies at 34 and 32 weeks of gestation, and they are both doing well. We therefore suggest possible mechanisms by which aggressive prophylactic factor support in a female with severe congenital coagulopathy, may improve on fetal outcome.
患有先天性凝血因子缺乏症的女性怀孕与不良胎儿结局有关。据报道有复发性自然流产、胎盘早剥和早产情况，但其确切原因尚不清楚，对此类患者的管理仍然是一项挑战。我们回顾了一名患有因子X缺乏症患者的四次妊娠结局，并观察了因子替代疗法对妊娠的影响。她的前两次妊娠分别在妊娠21周和25周时产下极早产婴儿，两个婴儿均在新生儿期死亡。在这些妊娠期间，该患者因急性出血发作接受了新鲜冰冻血浆治疗。此外，在她第二次怀孕时，她在妊娠后半期预防性地使用了因子IX复合物[康尼]。在接下来的两次妊娠中，她在妊娠早期就接受了治疗，预防性补充因子X。她分别在妊娠34周和32周时产下健康婴儿，两个婴儿目前情况良好。因此，我们提出了可能的机制，即对患有严重先天性凝血病的女性进行积极的预防性因子支持可能改善胎儿结局。
