Lapoirie Joëlle, Contis Anne, Guy Alexandre, Lifermann François, Viallard Jean-François, Sentilhes Loïc, James Chloé, Duffau Pierre
Department of Internal Medicine and Clinical Immunology, Hôpital Saint-André, CHU de Bordeaux, Bordeaux, France.
Laboratory of Hematology, Hôpital Haut-Lévèque, CHU de Bordeaux, Pessac, France.
J Matern Fetal Neonatal Med. 2020 Jan;33(1):49-56. doi: 10.1080/14767058.2018.1484097. Epub 2018 Jun 26.
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. 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. 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%. 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.
费城染色体阴性骨髓增殖性肿瘤（MPNs）在孕期会大幅增加母婴并发症的风险。目前，针对这类女性患者的管理缺乏国际共识。我们的研究旨在评估法国队列中MPN患者妊娠的当前管理情况及结局。我们回顾性分析了27例与特定突变相关的MPN女性患者的妊娠情况。确定了9例原发性血小板增多症女性患者的19次妊娠以及5例真性红细胞增多症女性患者的8次妊娠。我们的研究显示活产率为70%，但妊娠过程顺利的仅占30%。胎儿并发症主要为早期自然流产（22%）、胎儿生长受限（15%）和早产（15%）。母亲方面的问题包括血栓形成（15%）和出血（11%）。子痫前期和溶血、肝酶升高及血小板减少综合征的发生率较高（15%）。70%的妊娠进行了子宫动脉多普勒检查。43%的妊娠发现多普勒结果异常。血小板计数高且血细胞比容保持稳定或升高的妊娠以胎儿死亡和子宫胎盘功能障碍告终。根据专家共识，大多数妊娠（67%）可被归类为高危组，产科结局不良，标准风险妊娠中有50%结局顺利，而高危妊娠中这一比例为22%。72%的高危妊娠使用了肝素和/或干扰素α。这些妊娠的预后仍然很差，通过专家之间更有效的协作以及包括肝素和干扰素α在内的强化治疗，预后可能会得到改善。
