Paroxysmal nocturnal hemoglobinuria and pregnancy before the eculizumab era: the French experience
Paroxysmal nocturnal hemoglobinuria and pregnancy before the eculizumab era: the French experience
复制标题
DOI：
10.3324/haematol.2010.037531
复制
发表时间：
2011-09-01
期刊：
影响因子：
--
通讯作者：
Lamy, Thierry
中科院分区：
文献类型：
--
作者：
de Guibert, Sophie;de Latour, Regis Peffault;Lamy, Thierry
研究背景阵发性睡眠性血红蛋白尿症孕妇妊娠罕见，有关母婴死亡率的报道很少。设计与方法于2008年1月向法国血液学学会的所有成员发送了一份专门的问卷，旨在征集阵发性睡眠性血红蛋白尿症妇女的妊娠数据。诊断为阵发性睡眠性血红蛋白尿的中位年龄为21.5岁，怀孕时的中位年龄为27岁。这些妇女在怀孕期间都没有接受过eculizumab治疗。95%的病例出现母体并发症，主要是需要输血的红细胞减少症。记录了两例重型再生障碍性贫血(一例初发，另一例复发)。妊娠期未发生血栓事件，产后血栓形成4例(16%)，其中2例死亡(孕产妇死亡率8%)。大多数患者在孕期和产后接受了抗血栓治疗(n=16，%)。29%的病例是早产，53%的病例出生体重小于3公斤。胎儿死亡率为4%。结论妊娠合并阵发性睡眠性血红蛋白尿与母儿死亡率升高有关(在本组中分别为8%和4%)。产妇死亡率与产后血栓有关。建议在孕期和产后6周内预防性抗凝。
BackgroundPregnancy in women with paroxysmal nocturnal hemoglobinuria is rare, with few reports on maternal and fetal mortality rates.Design and MethodsA specific questionnaire designed to solicit data on pregnancies in women with paroxysmal nocturnal hemoglobinuria was sent to all members of the French Society of Hematology in January 2008.ResultsWe identified 27 pregnancies in 22 women at 10 French Society of Hematology centers between 1978 and 2008. The median age was 21.5 years at diagnosis of paroxysmal nocturnal hemoglobinuria and 27 years at pregnancy. None of these women had received eculizumab during their pregnancy. Maternal complications, consisting mostly of cytopenias requiring transfusions, occurred in 95% of cases. Two cases of severe aplastic anemia (de novo in one case and relapse in the other) were recorded. No thrombotic events occurred during pregnancy, whereas 4 postpartum thromboses (16%) were recorded, 2 of which were fatal (maternal mortality rate 8%). Most patients received antithrombotic prophylaxis during pregnancy and postpartum (n=16; 64%). Delivery was preterm in 29% of cases, and birth weight was less than 3 kg in 53% of cases. Fetal mortality rate was 4%.ConclusionsPregnancy during paroxysmal nocturnal hemoglobinuria is associated with increased maternal and fetal mortality rates (8% and 4%, respectively, in this series). Maternal mortality is related to postpartum thromboses. Prophylactic anticoagulation is recommended during pregnancy and for six weeks postpartum.