阵发性睡眠性血红蛋白尿症患者妊娠管理策略OA

Pregnancy in patients with Paroxysmal Nocturnal Hemoglobinuria(PNH)is considered high-risk,posing significant dangers to both the mother and the fetus.Major complications include aggravated hemolysis,thrombosis,infection,bleeding,preeclampsia,miscarriage,preterm birth,and fetal growth restriction.Before the era of complement inhibitors,maternal mortality rates ranged from 8％to 20％,and the rate of spontaneous abortion reached as high as 45％.In recent years,complement inhibitors,particularly eculizumab,have significantly improved pregnancy outcomes in PNH patients and have become the cornerstone of management.The core management strategy involves pre-conception assessment by a multidisciplinary team(MDT)including hematology,obstetrics,and anesthesiology.The ideal timing for pregnancy is when the disease is stable,with lactate dehydrogenase(LDH)levels below 1.5 times the upper limit of normal.During pregnancy,close monitoring of blood counts,LDH,and other indicators is required,along with a stratified management approach based on the degree of hemolysis:patients with significant hemolysis should initiate or continue complement inhibitor therapy promptly,with approximately 50％requiring dose escalation in the third trimester;those with low-level hemolysis can be closely monitored,with treatment initiated if necessary.All patients should receive thromboprophylaxis and complete meningococcal vaccination before starting complement inhibitor therapy.In conclusion,PNH is no longer an absolute contraindication to pregnancy.Through standardized,complement inhibitor-centered comprehensive management,multidisciplinary collaboration,and individualized treatment,maternal and fetal mortality can be significantly reduced,leading to improved pregnancy outcomes.