Maternidade Júlio Dinis, Centro Hospitalar do Porto, Porto, Portugal.
Eur Rev Med Pharmacol Sci. 2013 Nov;17(21):2857-9.
von Willebrand disease type 3, is an extremely rare condition. It can be severe and potentially life-threatening, particularly in pregnant women during labor and subsequently during early puerperium. Due to its rarity, there is no optimal treatment/management during pregnancy.
We describe two cases of pregnant women with von Willebrand disease type 3, and its successful surveillance and treatment with Haemate P FVIII (human plasma-derived von Willebrand Factor-ristocetin co-factor associated with human coagulation factor VIII), during pregnancy, partum and puerperium.
Daily prophylaxis with Haemate P FVIII in women with von Willebrand disease type 3, starting 2 hours before caesarean section until the 7th day of puerperium, associated with close analytical and clinical surveillance seems to be a safe clinical option.
血管性血友病 3 型是一种极为罕见的病症。该病可能十分严重,甚至危及生命,尤其是在孕妇分娩及随后的产褥期。由于其罕见性,目前尚无可用于妊娠期的最佳治疗/管理方法。
我们描述了两例血管性血友病 3 型孕妇的病例,她们在妊娠、分娩和产褥期使用 Haemate P FVIII(人血浆衍生的 von Willebrand 因子-瑞斯托菌素辅因子与人凝血因子 VIII 相关)进行成功的监测和治疗。
对于血管性血友病 3 型女性,在剖宫产前 2 小时开始每日预防性使用 Haemate P FVIII,直至产褥期第 7 天,并密切进行分析和临床监测,这似乎是一种安全的临床选择。