妊娠合并阵发性睡眠性血红蛋白尿4例及临床分析

Management of pregnancy with paroxysmal nocturnal hemoglobinuria:four cases report

目的探讨妊娠合并阵发性睡眠性血红蛋白尿(PNH)的临床特点、孕期处理及妊娠结局。方法回顾性分析2008年12月至2018年12月在我院收治的4例妊娠合并PNH患者的一般资料、孕期治疗方案及妊娠结局。结果(1)2例妊娠合并PNH患者在孕前发病并诊断为PNH,另2例患者在孕中期发病,其中1例产后才明确诊断为PNH;(2)孕前诊断的2例患者中,1例患者定期监测病情稳定后怀孕,孕晚期反复出现酱油色尿,对症支持维持至近足月;而另1例患者确诊后并未规律治疗,孕27周突发脾破裂伴感染导致晚期流产;孕期发病的2例患者均是孕中期出现血三系减低,1例患者确诊后因脾静脉血栓予低分子肝素抗凝治疗维持妊娠至足月;另1例患者孕晚期血三系减低逐渐加重,予丙种免疫球蛋白及氟美松治疗无效,对症维持至近足月终止妊娠,产后才确诊。(3)4例妊娠合并PNH患者中,1例患者孕中期病情进展导致晚期流产,其余3例均因血小板低在输注血小板后行择期剖宫产;3例新生儿中2例为医源性早产,其中1例因呼吸窘迫综合征转入新生儿监护病房,3例新生儿预后均良好。结论PNH患者在孕前需由血液科医师进行病情评估,病情稳定者可以妊娠;妊娠合并PNH患者应由血液病学家和专门从事高风险妊娠的产科医师共同进行孕期管理,以期获得良好的母胎结局。

Objective:To investigate the clinical features,management and outcome of pregnancy with paroxysmal nocturnal hemoglobinuria(PNH).Methods:The general information,pregnancy treatment and pregnancy outcome of four PNH patients with pregnancy in Peking Union Medical College Hospital from December 2008 to December 2018 were analyzed retrospectively.Results:(1)Two patients were diagnosed as PNH before pregnancy.The other two patients occurred in the second trimester,with one diagnosed after delivery.(2)Among the two patients diagnosed before pregnancy,one patient was regularly monitored and got pregnant after the condition stabilized.Soy sauce-colored urine appeared repeatedly in the third trimester,and symptomatic support was maintained until nearly full term.Another patient did not receive regular treatment after diagnosis,and suffered sudden splenic rupture with infection which led to late miscarriage.Both of the two patients with onset during pregnancy occurred pancytopenia in the second trimester,one patient received anticoagulation to maintain pregnancy to full-term.Another patient’three blood cell lines decreased of gradually in the third trimester of pregnancy.Her treatment with gamma immunoglobulin and flumethasone was ineffective.The symptomatic maintenance was maintained until the pregnancy was terminated near term,and the diagnosis was confirmed after delivery.(3)Among the four patients with PNH during pregnancy,one patient progressed in the second trimester leading to late miscarriage,and the remaining three patients underwent elective cesarean section after platelet transfusion due to low platelets.Two of the three neonates were iatrogenic preterm birth.One of the two premature infants was admitted to neonatal intensive care unit because of respiratory distress syndrome,and all the three newborns had good prognosis.Conclusions:Patients with PNH should have an overall evaluation before conception,and those with stable condition can be pregnant.Patients with PNH in pregnancy should be managed during pregnancy by hematologist and obstetrician who specializes in high-risk pregnancies in order to obtain a good maternal-fetal outcome.