The combined incidence of classical Philadelphia-negative myeloproliferative neoplasm (MPN) is 6-9/100 000 with a peak frequency between 50 and 70 years. MPN is less frequent in women of reproductive age. However, for essential thrombocythaemia (ET) in particular there is a second peak in women of reproductive age and 15% of polycythaemia vera (PV) patients are less than 40 years of age at the time of diagnosis. Thus these diseases are encountered in women of reproductive potential and may be diagnosed in pregnancy or in women being investigated for recurrent pregnancy loss. The incidence of MPN pregnancies is 3·2/100 000 maternities per year in the UK. The majority of data regarding Philadelphia-negative MPNs relates to patients with ET, for which the literature suggests significant maternal morbidity and poor fetal outcome; specifically maternal thrombosis and haemorrhage, miscarriage, pre-eclampsia, intrauterine growth restriction (IUGR), stillbirth and premature delivery as summarised in the recent systematic review and meta-analysis in Blood, 2018, 132, 3046. The literature for PV is more sparse but increasing and is concordant with ET pregnancy outcomes. The literature regarding primary myelofibrosis (PMF) is even more scarce. Treatment options include aspirin, venesection, low molecular weight heparin (LMWH) and cytoreductive therapy. Data and management recommendations are often extrapolated from other pro-thrombotic conditions or from ET to PV and PMF. Women of reproductive age with a diagnosis of MPN should receive information and assurance regarding management and outcome of future pregnancies. From pre-conceptual planning to the post-partum period, women should have access to joint care from an obstetrician with experience of high-risk pregnancies and a haematologist in a multidisciplinary setting. This paper provides an update with regards to Philadelphia-negative MPN in pregnancy, details local practise in an internationally recognised centre for patients with MPN and outlines a future research strategy.

中文翻译：

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我们如何处理妊娠期费城阴性的骨髓增生性肿瘤。

经典费城阴性骨髓增生性肿瘤（MPN）的合并发生率为6-9 / 100 000，峰值频率在50到70岁之间。育龄妇女的MPN发病率较低。但是，对于原发性血小板增多症（ET），尤其是育龄妇女出现了第二高峰，诊断时，15％的真性红细胞增多症（PV）患者年龄不到40岁。因此，这些疾病在具有生殖潜能的妇女中遇到，可以在妊娠中或正在接受反复性妊娠损失调查的妇女中进行诊断。在英国，MPN怀孕的发生率每年为3·2/100 000。费城阴性MPNs的大多数数据与ET患者有关，文献表明，母亲的发病率很高，胎儿结局也很差。如最近对血液的系统综述和荟萃分析（2018，132，3046）中所概述的，尤其是母亲血栓形成和出血，流产，先兆子痫，子宫内生长受限（IUGR），死胎和早产。PV文献较为稀疏但增加并与ET妊娠结局一致。关于原发性骨髓纤维化（PMF）的文献更加稀少。治疗选择包括阿司匹林，穿刺术，低分子量肝素（LMWH）和细胞减少疗法。数据和管理建议通常是从其他血栓形成前状况或从ET推算为PV和PMF。诊断为MPN的育龄妇女应获得有关妊娠管理和结果的信息和保证。从概念前的计划到产后，妇女应从具有高风险怀孕经验的妇产科医生和多学科环境的血液科医生那里获得联合护理。本文提供了有关费城阴性MPN孕妇的最新信息，详细介绍了国际公认的MPN患者中心的本地实践，并概述了未来的研究策略。