Maternal sickle cell disease and twin pregnancy: a case series and review of the literature

Hematology. 2019 Dec;24(1):148-158. doi: 10.1080/10245332.2018.1535534. Epub 2018 Oct 21.

Abstract

Objectives: Maternal sickle cell disease (SCD) and multiple gestations are well known separately as causes of high-risk pregnancies, however, there is sparse information available on maternal and perinatal outcome when both conditions occur together. This case series describes the outcomes of women with maternal SCD and twin pregnancy in the largest single-center case series to date.

Methods: Retrospective identification of all twin pregnancies in maternal SCD patients between 2006 and 2016 at Guy's and St. Thomas' Hospital, United Kingdom Results: Eight women were included: seven with HbSS and one with HbSC. Our cohort experienced common SCD-related and pregnancy-related complications such as painful vaso-occlusive crises (VOC), acute chest syndrome (ACS), and pre-eclampsia and less common complications such as peri-partum cardiomyopathy and delayed hemolytic transfusion reaction. Only two out of eight women had relatively uncomplicated pregnancies. Seven out of eight women required transfusion antenatally and there was no maternal or perinatal mortality. A review of the available literature highlighted the lack of available information on this uncommon cohort. It was evident that outcomes have improved over the years, where historical studies demonstrate higher rates of maternal and perinatal mortality.

Discussion: The antenatal and postnatal complications described in our study and literature review highlights the significant morbidity and mortality associated with these high-risk pregnancies.

Conclusion: Our case series highlights the advantage of closer monitoring and comprehensive multidisciplinary care in delivering improved clinical outcomes.

Keywords: Sickle cell; anemia; transfusion; twin pregnancy.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Acute Chest Syndrome / pathology
  • Acute Chest Syndrome / physiopathology
  • Acute Chest Syndrome / therapy*
  • Adult
  • Blood Transfusion*
  • Female
  • Humans
  • Pre-Eclampsia / pathology
  • Pre-Eclampsia / physiopathology
  • Pre-Eclampsia / therapy*
  • Pregnancy
  • Pregnancy Complications, Hematologic / pathology
  • Pregnancy Complications, Hematologic / physiopathology
  • Pregnancy Complications, Hematologic / therapy*
  • Pregnancy, Twin*