Michiels J J, Hamulyak K, Nieuwenhuis H K, Novakova I, van Vliet H H
Haematology, Haemostasis and Thrombosis Scientific Center, Goodheart Institute Rotterdam, The Netherlands.
Eur J Haematol. 1997 Aug;59(2):105-9. doi: 10.1111/j.1600-0609.1997.tb00733.x.
The present study reports on the treatment of bleeding episodes and the natural history of factor VIII inhibitors in 4 patients with acquired haemophilia A postpartum. Low titre type II factor VIII inhibitors in 3 patients and high titre type I inhibitor in 1 patient became apparent immediately to 7 months after delivery. High dose human factor VIII concentrate substitution was effective in controlling bleeding episodes in two cases of factor VIII inhibitor type II, but ineffective in 1 patient with high titre type I factor VIII inhibitor. High dose gammaglobulin intravenously in 1 patient with type II factor VIII inhibitor induced a partial correction of factor VIIIc levels for 2 wk. Immunosuppressive treatment in all 4 patients with acquired haemophilia A postpartum did not reduce the potency of the factor VIII inhibitors. The low titre type II inhibitors spontaneously disappeared in all 3 patients within a few months to 1 yr after discontinuation of the immunosuppressive treatment. The high titre type I factor VIII inhibitor persisted for more than 24 yr. We conclude that immunosuppression in 4 women with acquired haemophilia A postpartum did not significantly affect the factor VIII inhibitor titre.
本研究报告了4例产后获得性血友病A患者出血事件的治疗情况及Ⅷ因子抑制剂的自然病程。3例患者出现低滴度Ⅱ型Ⅷ因子抑制剂,1例患者出现高滴度Ⅰ型抑制剂,这些情况在产后立即至7个月时显现出来。高剂量人Ⅷ因子浓缩物替代疗法对2例Ⅱ型Ⅷ因子抑制剂患者控制出血事件有效,但对1例高滴度Ⅰ型Ⅷ因子抑制剂患者无效。1例Ⅱ型Ⅷ因子抑制剂患者静脉注射高剂量丙种球蛋白使Ⅷ因子c水平部分纠正了2周。所有4例产后获得性血友病A患者的免疫抑制治疗均未降低Ⅷ因子抑制剂的效力。3例低滴度Ⅱ型抑制剂患者在停用免疫抑制治疗后的数月至1年内均自发消失。高滴度Ⅰ型Ⅷ因子抑制剂持续存在超过24年。我们得出结论,4例产后获得性血友病A女性患者的免疫抑制治疗对Ⅷ因子抑制剂滴度无显著影响。