Lévesque H, Guillet B, Benhamou Y
Service de médecine interne, Normandie université, UNIROUEN, Interne, 76031 Rouen, France.
Centre de traitement des maladies hémorragiques, CHU de Rennes, Rennes, France; Inserm, EHESP, IRSET (institut de recherche en santé, environnement et travail) - UMR_S 1085, Université Rennes, CHU de Rennes, 35000 Rennes, France.
Rev Med Interne. 2022 Aug;43(8):494-497. doi: 10.1016/j.revmed.2022.04.012. Epub 2022 May 27.
The occurrence of acquired hemophilia during pregnancy or postpartum is rare (2 to 10 % in series). It is generally suspected in the presence of haemorrhagic manifestations (especially subcutataneous or mucosal bleeding) associated with an isolated prolongation of the activated partial thromboplastin time (APTT). The diagnosis is confirmed by the association of a low level of factor VIII (FVIII) and the presence of an anti-FVIII inhibitor. Postpartum management is similar to that of other acquired haemophilias: correction of a severe haemorrhagic syndrome by "bypassing" agents, eradication of the inhibitor by corticosteroids alone or in combination with another immunosuppressive agent depending on the residual level of FVIII and the titer of the inhibitor. Management of the forms occurring during pregnancy is based on rare experiences or expert opinions. The management of childbirth is particularly delicate in terms of haemorrhage, especially if the anti-FVIII inhibitor is still present, and must be prepared in a multidisciplinary manner. Finally, as with any acquired hemophilia, a relapse is possible, especially in the year following remission. During a subsequent pregnancy, the risk of recurrence is possible but should not be a contraindication to a new pregnancy.
妊娠期间或产后发生获得性血友病较为罕见(系列报道中占2%至10%)。通常在出现出血表现(尤其是皮下或黏膜出血)且活化部分凝血活酶时间(APTT)单独延长时怀疑该病。通过低水平的凝血因子VIII(FVIII)以及存在抗FVIII抑制剂可确诊。产后管理与其他获得性血友病相似:通过“旁路”药物纠正严重出血综合征,根据FVIII残留水平和抑制剂滴度,单独使用皮质类固醇或与另一种免疫抑制剂联合使用以清除抑制剂。孕期发生的病例管理基于罕见的经验或专家意见。分娩管理在出血方面尤其棘手,特别是如果抗FVIII抑制剂仍然存在,必须以多学科方式做好准备。最后,与任何获得性血友病一样,可能会复发,尤其是在缓解后的一年内。在随后的妊娠期间,复发风险是可能存在的,但不应成为再次妊娠的禁忌证。