妊娠合并慢性骨髓增殖性疾病11例临床分析

Clinical analysis of eleven patients with chronic myeloproliferative disorders complicating pregnancy

目的 探对妊娠合并慢性骨髓增殖性疾病（CMPD）的临床特点及处理、妊娠结局及预后.方法 回顾性分析北京大学人民医院妇产科2000-2009年收治的11例妊娠合并CMPD患者[其中原发性血小板增多症（ET）5例,原发性骨髓纤维化（PMF）1例,慢性粒细胞白血病（CML）5例]的临床资料.结果 11例妊娠合并CMPD患者共妊娠12例次.（1）有规律产前检查者5例次,均行定期血常规等检查,适当给予抗凝治疗,预防并发症发生.其中1例PMF患者为孕前诊断,初次妊娠至32周时发生轻度子痫前期及胎死宫内;2年后再次妊娠,孕早期筛查抗β2糖蛋白Ⅰ抗体升高,给予小剂量阿司匹林口服及低分子肝素抗凝治疗,孕期顺利、足月分娩.（2）无规律产前检查者5例次,其中ET患者2例次,CML患者3例次.1例ET患者于妊娠25周发生重度子痫前期,脐动脉舒张期血流反向,经解痉、降压及抗凝治疗效果不佳,因血压进行性升高、胎盘早剥而紧急行剖官产术终止妊娠,胎儿娩出即死亡.从未产前检查者2例次,均因临产入院,诊断为CML.（3）合并羊水过少4例次,子痫前期3例次,其中重度子痫前期2例次,轻度子痫前期1例次,所有患者均无严重出血及血栓形成等并发症发生.（4）妊娠至足月者8例次,其中4例次剖宫产分娩;4例次阴道分娩.早产2例次,均因羊水进行性减少以剖宫产术终止妊娠.分娩的10例新生儿出生体质量1820～3600 g,除1例并发重度子痫前期者出现胎儿生长受限（FGR）外,其余均为适于胎龄儿.（5）11例患者妊娠期间原有疾病均病情稳定,其中3例CML患者妊娠晚期口服羟基脲治疗,4例ET患者及1例CML患者终止妊娠前进行血小板单采术治疗,效果良好.除1例CML患者分娩后5个月因疾病进展死亡外,余患者产后病情均平稳.结论 妊娠合并CMPD患者多数妊娠结局良好,孕期管理需警惕血栓形成、子痫前期、胎死官内、FGR等并发症.合理应用抗凝等治疗,有助改善母儿结局.

Objective To investigate the clinical characteristics, the antenatal management, the outcome and prognosis of chronic myeloproliferative disorders （CMPD） complicating pregnancy. Methods Retrospectively analyze the clinical data of eleven patients with CMPD complicating pregnancy hospitalized in Peking University People＇ s Hospital from 2000 to 2009, including five patients with essential thrombocythemia, one with primary myelofibrosis and five with chronic myeloid leukemia. Results （1）Five pregnancies had periodic antenatal care and laboratory monitorings like full blood count. Reasonable anti-coagulation therapy was given to prevent the complications. One patient with PMF diagnosed before conception had her first pregnancy ended with mild pre-eclampsia and intrauterine death at the gestational age of 32 weeks. During the first trimester of her second pregnancy two years later, the test for anti-β2 glycoprotein antibody was positive. She received low-dose aspirin and low-molecular-weight heparin as anticoagulants. An uneventful course was obtained and she delivered a healthy term infant. （2） Five pregnancies had occasional antenatal examination, including two patients with ET and three patients with CML One patient with ET developed severe pre-eclampsia at the gestational age of 25 weeks. Umbilical artery Doppler showed reversed end-diastolic velocity. The management with anti-convulsants, antihypertensives and anti-coagulants showed no effect. An emergency cesarean section had to be performed because of the aggressive hypertension and placental abruption, with still birth as a result. Two pregnancies never had an antenatal care. Both of them were admitted on labor and the diagnoses of CML were made. （3）Four pregnancies developed oligohydramnios and three developed preelampsia（two severe pre-eclampsia and one mild pre-eclampsia）. There was no other hemorrhage and thrombosis event. （4） Eight pregnancies reached full-term with four cesarean sections and four vaginal births. Two preterm cesarean sections were performed because of a progressive oligohydramnios. The ten live neonates weighed 1820 - 3600 g. All were appropriate for gestational age, except one fetal growth retardation （FGR） developed in one patient with severe pre-eclampsia. （5） As for the CMPD, the eleven patients were all in stable conditions. Three patients with CML received hydroxyurea in the third trimester, four with ET and one with CML had plateletpheresis before delivery with favorable effect. All patients were uneventful postpartum, except one with CML who died in 5 months after childbirth. Conclusions The pregnancy outcomes for patients with CMPD are mostly good. However, antenatal care should pay more attention to the complications such as thromboembolic accidents, pre-eclampsia, still birth and fetal growth retardation. Management including reasonable anticoagulation therapy should be considered, which may help improve the prognosis.