Coller B S, Hultin M B, Hoyer L W, Miller F, Dobbs J V, Dosik M H, Berger E R
Blood. 1981 Sep;58(3):619-24.
A 22-yr-old primigravada developed a hemorrhagic diathesis 6 days after delivering a normal female infant and was found to have an immunoglobulin inhibitor of factor VIII (15 Bethesda units). The patient was treated with prednisone and the bleeding stopped soon thereafter. Her inhibitor titer decreased over the next 8 mo, at which time no inhibitor was detectable. Nine months later she became pregnant again and proceeded to have an uneventful pregnancy and delivery of a normal female infant without evidence of a recurrence of the inhibitor. Studies of 3H-thymidine incorporation into the patient's lymphocytes in the presence of her own plasma or plasmas from her husband, normals, a von Willebrand patient, and a hemophilic patient yielded equivocal results. Analysis of VIII:CAg using 2 different antisera failed to discern immunologic differences between the VIII:CAg of the patient, her husband, or her first child. A review of the literature revealed that there were no recurrences with second pregnancies in any of the 8 patients with postpartum factor VIII inhibitors whose inhibitors had completely disappeared prior to delivery. While the pathogenesis of this disorder remains uncertain, the apparently favorable prognosis for such patients should be considered in counselling with regard to future pregnancies.
一名22岁初产妇在分娩一名正常女婴6天后出现出血素质,发现其存在因子VIII免疫球蛋白抑制剂(15贝塞斯达单位)。患者接受泼尼松治疗,此后出血很快停止。其抑制剂滴度在接下来的8个月内下降,此时已检测不到抑制剂。9个月后她再次怀孕,孕期顺利,分娩出一名正常女婴,且无抑制剂复发迹象。在患者自身血浆或其丈夫、正常人、血管性血友病患者及血友病患者的血浆存在的情况下,对患者淋巴细胞中3H-胸腺嘧啶核苷掺入情况的研究结果不明确。使用2种不同抗血清对VIII:CAg进行分析,未能辨别出该患者、其丈夫或其第一个孩子的VIII:CAg之间的免疫学差异。文献回顾显示,8例产后因子VIII抑制剂在分娩前抑制剂已完全消失的患者中,第二次怀孕均未复发。虽然该疾病的发病机制仍不确定,但在为这类患者提供关于未来妊娠的咨询时,应考虑到其明显良好的预后。