妊娠合并阵发性睡眠性血红蛋白尿患者的临床分析并文献复习  

Clinical analysis of patients with pregnancy complicating paroxysmal nocturnal hemoglobinuria and literature review

作　　者：姜亚非 冯周善 王亚菲 聂庆文 贺芳[1] JIANG Yafei;FENG Zhoushan;WANG Yafei;NIE Qingwen;HE Fang(Guangdong Provincial Key Laboratory of Obstetrics and Gynecology Major Diseases/Guangdong Provincial Clinical Medical Research Center for Obstetric and Gynecologic Diseases/Guangdong Hong Kong Macao Joint Laboratory of Maternal and Fetal Medicine/Department of Obstetrics and Gynecology,Third Affiliated Hospital of Guangzhou Medical University,Guangzhou,Guangdong 510145,China;Department of Obstetrics,Guiyang Municipal Second People’s Hospital,Guiyang,Guizhou 550005,China)

机构地区：[1]广东省产科重大疾病重点实验室/广东省妇产疾病临床医学研究中心/粤港澳母胎医学高校联合实验室/广州医科大学附属第三医院妇产科,广州510145 [2]贵阳市第二人民医院产科,贵阳550005

出　　处：《重庆医学》2025年第1期142-147,共6页Chongqing Medical Journal

摘　　要：目的探讨妊娠合并阵发性睡眠性血红蛋白尿(PNH)患者的临床特点及研究进展。方法回顾性分析2009年9月至2023年1月广州医科大学附属第三医院收治的10例次妊娠合并PNH患者的临床资料,并以“paroxysmal nocturnal hemoglobinuria”和“pregnancy”为英文关键词在PubMed及以“阵发性睡眠性血红蛋白尿”和“妊娠”为中文关键词在中国知网和万方数据库检索1986年1月至2022年12月收录的文献,对回顾性分析的10例次及检索报道的33例次共43例次妊娠合并PNH患者的临床特点、孕期诊疗及妊娠结局采用描述性统计方法进行分析。结果43例次妊娠中,常见临床症状贫血29例次(67.44%)、血小板减少17例次(39.53%)、酱油色尿15例次(34.88%)、疲劳6例次(13.95%)、腹痛5例次(11.63%)、孕产期发生血栓8例次(18.60%)、并发子痫前期10例次(23.25%)。31例次(72.09%)输注红细胞,11例次(25.58%)输注血小板,19例次(44.18%)使用糖皮质激素、25例次(58.13%)使用低分子肝素(LMWH)治疗。胎儿生长受限11例次(25.58%);早产17例次(39.53%),其中早期早产6例次(28~33+6周),早期早产率13.95%,晚期早产11例次(34~36+6周),晚期早产率25.58%;死胎3例次(6.97%)。结论妊娠合并PNH患者的母儿并发症与不良结局的发生率较高,应在综合医院由血液科医师和具有处理高危妊娠经验的产科医师共同管理。Objective To investigate the clinical characteristics and research progress of the patients with pregnancy complicating paroxysmal nocturnal hemoglobinuria(PNH).Methods The clinical data of 10 case-times of pregnancy complicating PNH admitted and treated in the Third Affiliated Hospital of Guangzhou Medical University from September 2009 to January 2023 were retrospectively analyzed.“Paroxysmal nocturnal hemoglobinuria”and“pregnancy”served as the English keywords,the literatures included in PubMed were retrieved.“paroxysmal nocturnal hemoglobinuria”and“pregnancy”served as the Chinese keywords,the literatures included in CNKI and Wan Fang Databases from January 1986 to December 2022 were retrieved.The clinical characteristics,pregnancy diagnosis and treatment,and pregnancy outcome of together 43 case-times pregnancies complicating with PNH in 10 case-times by retrospective analysis and 33 case-times by retrieval report were analyzed by descriptive statistic method.Results Among 43 case-times of pregnancy,the common clinical symptoms had 29 case-times of anemia(67.44%),17 case-times of thrombocytopenia(39.53%),15 case-times of soy sauce color urine(34.88%),6 case-times of fatigue(13.95%),5 case-times of abdominal pain(11.63%),8 case-times of thrombosis(18.60%)during pregnancy and childbirth and 10 cases of complicating preeclampsia(23.25%).There were 31 case-times(72.09%)of red blood cell transfusions and 11 case-times(30.23%)of platelet transfusions;19 case-times(48.83%)of glucocorticoids use and 25 case-times(46.51%)of low molecular weight heparin(LMWH)use.There were 11 case-times(25.58%)of fetal growth restriction,17 case-times of premature delivery(39.53%),in which 6 case-times(28-33^(+6)weeks)of early stage premature delivery,the early premature delivery rate was 13.95%,there were 11 case-times of late premature delivery(34-36+6 weeks),the rate of late premature delivery was 25.58%.There were 3 case-times(6.97%)of stillbirth.Conclusion The incidence rate of mother and infant complications and a

关 键 词：阵发性睡眠性血红蛋白尿 妊娠并发症 妊娠结局 围生期管理 临床特点 

分 类 号：R714.254[医药卫生—妇产科学]