Department of Medicine, Division of Hematology, Western University, London, Ontario, Canada.
Department of Obstetrics & Gynecology, Western University, London, Ontario, Canada.
Transfusion. 2020 Oct;60(10):2448-2455. doi: 10.1111/trf.16003. Epub 2020 Aug 26.
Sickle cell disease (SCD) is associated with hematologic complications including delayed hemolytic transfusion reactions (DHTRs) and pregnancy-related morbidity and mortality. Hyperhemolysis syndrome (HS) is the most severe form of DHTR in patients with SCD, in which both transfused and native red blood cells are destroyed. Further transfusions are avoided after a history of HS. Immunosuppressive agents can be used as prophylaxis against life-threatening hemolysis when transfusion is necessary. There is a paucity of evidence for the use of HS prophylaxis before transfusions, the continuation of hydroxyurea (HU) in lieu of chronic transfusion, and the use of erythropoiesis-stimulating agents (ESA) in pregnant SCD patients.
We present a case of a pregnant patient with SCD and a previous history of HS. HS prophylaxis was given before transfusion with corticosteroids, intravenous immunoglobulin, and rituximab. In addition, HU was continued during pregnancy to control SCD, along with the use of concomitant ESA to maintain adequate hemoglobin levels and avoid transfusion. We describe a multidisciplinary approach to pregnancy and delivery management including tailored anesthetic and obstetric planning.
This is the first published case of HS prophylaxis in a pregnant SCD patient, with good maternal and fetal outcomes after transfusion. HU and ESAs were able to control SCD and mitigate anemia in lieu of prophylactic transfusions during pregnancy. Further prospective studies are necessary to elucidate the ideal management of pregnant SCD patients with a history of HS or other contraindications to chronic transfusion.
镰状细胞病（SCD）与血液学并发症相关，包括延迟性溶血性输血反应（DHTR）以及与妊娠相关的发病率和死亡率。高血红蛋白血症综合征（HS）是 SCD 患者中最严重的 DHTR 形式，其中输注的和自身的红细胞均被破坏。在发生 HS 病史后，避免进一步输血。当需要输血时，免疫抑制剂可作为预防危及生命的溶血的预防措施。在输血前使用 HS 预防、替代慢性输血使用羟基脲（HU）以及在妊娠 SCD 患者中使用促红细胞生成素刺激剂（ESA）方面，证据不足。
我们报告了一例患有 SCD 且有 HS 病史的妊娠患者。在输血前使用皮质类固醇、静脉注射免疫球蛋白和利妥昔单抗进行 HS 预防。此外，HU 在怀孕期间继续使用以控制 SCD，并同时使用 ESA 维持足够的血红蛋白水平并避免输血。我们描述了一种多学科方法来管理妊娠和分娩，包括量身定制的麻醉和产科计划。
这是首例在妊娠 SCD 患者中进行 HS 预防的发表病例，输血后母婴结局良好。HU 和 ESA 能够控制 SCD 并减轻贫血，而无需在妊娠期间进行预防性输血。需要进一步的前瞻性研究来阐明有 HS 病史或其他慢性输血禁忌的妊娠 SCD 患者的理想管理。
