Management and outcomes of 27 pregnancies in women with myeloproliferative neoplasms

Introduction: Philadelphia-negative myeloproliferative neoplasms (MPNs) greatly increase the risk of maternal and fetal complications during pregnancy. Currently, international agreements regarding the management of these women are lacking. Patients and methods: Our study aimed to assess the current management and outcomes of MPN pregnancies in a French cohort. We retrospectively analyzed 27 pregnancies in women with MPNs that were associated with a specific mutation. Nineteen pregnancies in nine women with essential thrombocythemia and eight pregnancies in five women with polycythemia vera were identified. Results: Our study showed 70% live births, but only 30% uneventful pregnancies. Fetal complications were mainly early spontaneous abortions (22%), fetal growth restriction (15%), and premature delivery (15%). Maternal issues were divided between thrombosis (15%) and hemorrhages (11%). High rates of preeclampsia and hemolysis, elevated liver enzymes, and low platelet count syndrome (15%) were reported. Uterine artery Doppler was performed in 70% pregnancies. Abnormal Doppler results were found in 43% pregnancies. Pregnancies with high platelet counts and packed cell volume remaining static or increasing ended with fetal death and utero-placental dysfunction. According to expert consensus, most of the pregnancies (67%) could be stratified in the high risk group and had a bad obstetrical outcome, with 50% standard-risk pregnancies versus 22% high-risk pregnancies that were uneventful. Higher risk pregnancies were prescribed heparin and/or interferon α in 72%. Conclusions: The prognosis of these pregnancies remains very bad and may be improved by a more effective collaboration between specialists as well as a therapeutic intensification including heparin and interferon α.引言：费城染色体阴性骨髓增殖性肿瘤（Philadelphia-negative myeloproliferative neoplasms, MPNs）可显著增加妊娠期孕产妇与胎儿的并发症风险。目前，此类患者的临床管理暂无国际统一共识。 患者与方法：本研究旨在评估法国队列中合并MPNs的妊娠患者的当前诊疗现状与妊娠结局。我们回顾性分析了27例携带特定突变的MPNs女性的妊娠病例，其中9例原发性血小板增多症（essential thrombocythemia）女性共19次妊娠，5例真性红细胞增多症（polycythemia vera）女性共8次妊娠。 结果：本研究显示，该队列活产率为70%，但仅30%的妊娠过程平稳无并发症。胎儿并发症主要包括早期自然流产（22%）、胎儿生长受限（15%）及早产（15%）；孕产妇并发症则分为血栓形成（15%）与出血（11%）两类。此外，子痫前期及溶血、肝酶升高、血小板减少综合征（hemolysis, elevated liver enzymes, and low platelet count syndrome, HELLP综合征）的发生率达15%。70%的妊娠患者接受了子宫动脉多普勒超声检查，其中43%的检查结果异常。血小板计数及红细胞比容持续升高或维持高水平的妊娠，最终以胎儿死亡及子宫胎盘功能不全告终。根据专家共识，67%的妊娠可被划分为高危组，产科结局较差；标准风险组妊娠中仅50%过程平稳，而高危组仅22%的妊娠无并发症发生。72%的高危妊娠患者接受了肝素和/或α干扰素治疗。 结论：此类妊娠的预后仍较差，通过多学科专家间更高效的协作，以及采用包含肝素与α干扰素的强化治疗方案，或可改善其妊娠结局。