妊娠合并阵发性睡眠性血红蛋白尿患者的母儿结局  

Perinatal outcomes of paroxysmal nocturnal hemoglobinuria during pregnancy: cases report and literature review

作　　者：崔舒珲 杨京晶[1] 梁梅英[1] 张晓红[1] Cui Shuhui;Yang Jingjing;Liang Meiying;Zhang Xiaohong(Department of Obstetrics and Gynecology,Peking University People's Hospital,Beijing 100044,China)

机构地区：[1]北京大学人民医院妇产科,100044

出　　处：《中华围产医学杂志》2021年第12期911-916,共6页Chinese Journal of Perinatal Medicine

摘　　要：目的探讨妊娠合并阵发性睡眠性血红蛋白尿(paroxysmal nocturnal hemoglobinuria,PNH)患者的病情变化及治疗。方法回顾性分析2010年9月至2020年9月北京大学人民医院产科收治的9例妊娠合并PNH患者的临床资料,分析妊娠合并PNH患者的病情变化、治疗方法、母儿并发症及结局。对数据采用描述性统计方法进行分析。结果9例患者中,6例为经典型,3例合并骨髓衰竭性疾病。孕期接受输血支持/小剂量糖皮质激素或联合治疗者共8例,其中同时行抗凝治疗者4例。8例孕前诊断PNH者中,7例妊娠期病情加重。8例发生妊娠并发症(包括胎儿生长受限7例、妊娠期高血压疾病和早产各4例、血栓和胎儿宫内死亡各1例)。9例患者均存活,其中8例妊娠至分娩,分娩孕周为37周(33~38周)。产后随访50月(4~92月),患者病情稳定,子代生长发育未发现异常。结论PNH患者孕期病情易加重,母儿并发症及不良结局发生率升高。多学科联合管理可能有助于改善母儿结局。Objective To explore the progression of paroxysmal nocturnal hemoglobinuria(PNH)during pregnancy and treatment for improving maternal and infant outcomes.Methods Nine pregnant women with PNH were admitted to the Obstetrics Department of Peking University People's Hospital from September 2010 to September 2020.The clinical data of these patients were retrospectively collected and analyzed.Relevant literature was reviewed to summarize the progression,treatment,complications,perinatal outcomes,and follow-up of PNH during pregnancy.Descriptive methods were used for statistical analysis.Results Among the nine patients,six were classic PNH,and three combined with bone marrow failure disease.Eight cases received blood transfusion/low-dose corticosteroids or combination therapy during pregnancy,and four of them were also received anticoagulants.In seven out of the eight patients diagnosed prenatally,the disease worsened during pregnancy.Complications were noted in eight patients during pregnancy,including fetal growth restriction in seven cases,hypertension and premature delivery in four cases each,thrombosis and intrauterine fetal death in one case each.No maternal deaths were reported,with a live birth rate of 8/9 between 33-38 gestational weeks,with the median at 37 weeks.The median time of postpartum follow-up was 50 months(4-92 months),during which the patients'conditions were all stable,and no abnormalities were found in the growth and development of the babies.Conclusions Pregnant women with PNH tend to worsen with an increased incidence of perinatal complications and adverse outcomes.Multidisciplinary management is recommended for this population may help improve maternal and infant outcomes.

关 键 词：血红蛋白尿 阵发性 妊娠并发症 血液 胎儿生长迟缓 妊娠结局 

分 类 号：R714.254[医药卫生—妇产科学]