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早期预防性红细胞单采术治疗镰状细胞病女性患者可改善母婴结局。

Improvement of maternal and fetal outcomes in women with sickle cell disease treated with early prophylactic erythrocytapheresis.

作者信息

Vianello Alice, Vencato Elisa, Cantini Maurizio, Zanconato Giovanni, Manfrin Erminia, Zamo Alberto, Zorzi Francesco, Mazzi Filippo, Martinelli Nicola, Cavaliere Elena, Monari Francesca, Venturelli Donatella, Ferrara Francesca, Olivieri Oliviero, De Franceschi Lucia

机构信息

Department of Medicine, University of Verona and AOUI-Verona, Policlinico GB Rossi, Verona, Italy.

Department of Transfusion Medicine, AOUI-Verona, Verona, Italy.

出版信息

Transfusion. 2018 Sep;58(9):2192-2201. doi: 10.1111/trf.14767. Epub 2018 Jul 8.

Abstract

BACKGROUND

The desire for pregnancy in sickle cell disease (SCD) women has become a true challenge for hematologists, requiring a multidisciplinary approach. Erythrocytapheresis (ECP) is an important therapeutic tool in SCD, but only limited data on starting time and the effects of ECP during pregnancy are available.

STUDY DESIGN AND METHODS

This is a double-center retrospective cross-sectional study on a total of 46 single pregnancies in SCD women from January 2008 to June 2017. ECP was started at 10.7 ± 5.2 weeks of gestation, and prophylactic enoxaparin (4,000 U daily) was introduced due to the reported high prevalence of thromboembolic events in pregnant SCD women.

RESULTS

The alloimmunization ratio was 2.1 per 1,000 and the alloimmunization rate was 5.6%. In early ECP-treated SCD women, no severe vaso-occlusive crisis, sepsis or severe infection, or preeclampsia or eclampsia were observed. We found normal umbilical arterial impedance during pregnancy, suggesting an optimal uteroplacental function in early ECP-treated SCD women. This was also supported by the improvement in newborn birthweights compared to previous studies. In our cohort, three SCD women were started later on ECP (20-25 weeks), and gestation ended with late fetal loss. Placenta pathology documented SCD-related damage and erythroblasts in placental vessels, indicating fetal hypoxia.

CONCLUSIONS

Collectively, our data generate a rationale to support a larger clinical trial of early ECP program in SCD pregnancy.

摘要

背景

镰状细胞病(SCD)女性的妊娠意愿已成为血液科医生面临的一项真正挑战,需要多学科方法。红细胞单采术(ECP)是SCD中的一种重要治疗工具,但关于妊娠期间开始时间和ECP效果的可用数据有限。

研究设计与方法

这是一项双中心回顾性横断面研究,共纳入2008年1月至2017年6月期间46例SCD女性的单胎妊娠。ECP在妊娠10.7±5.2周开始,由于报道妊娠SCD女性血栓栓塞事件的高发生率,引入了预防性依诺肝素(每日4000 U)。

结果

同种免疫率为每1000例中有2.1例,同种免疫发生率为5.6%。在早期接受ECP治疗的SCD女性中,未观察到严重的血管闭塞性危机、败血症或严重感染、先兆子痫或子痫。我们发现妊娠期间脐动脉阻抗正常,表明早期接受ECP治疗的SCD女性子宫胎盘功能最佳。与先前研究相比,新生儿出生体重的改善也支持了这一点。在我们的队列中,3例SCD女性较晚开始ECP治疗(20 - 25周),妊娠以晚期胎儿丢失告终。胎盘病理学记录了SCD相关损伤和胎盘血管中的成红细胞,表明胎儿缺氧。

结论

总体而言,我们的数据为支持在SCD妊娠中进行早期ECP方案的更大规模临床试验提供了理论依据。

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