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Sickle cell E hemoglobinopathy and pregnancy.

A J Ramahi1, L M Lewkow, M P Dombrowski

  • 1Department of Obstetrics and Gynecology, Hutzel Hospital/Wayne State University, Detroit, Michigan.

Obstetrics and Gynecology
|March 1, 1988
PubMed
Summary
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This case report details the first pregnancy with hemoglobin S/E, a rare hemoglobinopathy. Management involved exchange transfusion, highlighting potential perinatal risks but also favorable outcomes for certain hemoglobin E variants.

Area of Science:

  • Hematology
  • Obstetrics
  • Genetics

Background:

  • Increasing incidence of E hemoglobinopathies in the United States.
  • Hemoglobin S/E is a rare hemoglobinopathy, with limited data on its presentation in pregnancy.

Observation:

  • First case report of hemoglobin S/E in pregnancy.
  • Management strategy mirrored that of hemoglobin S/beta-thalassemia, utilizing partial exchange transfusion.
  • Observed complications included fetal distress, congenital hydrocephalus, and cleft lip and palate.

Findings:

  • Hemoglobin S/E in pregnancy presents unique challenges and potential complications.
  • Partial exchange transfusion was employed as a management strategy.
  • Pregnancy outcomes for S/E and E/beta-thalassemia hemoglobinopathies may involve increased perinatal risks.

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Implications:

  • This case highlights the need for careful monitoring and management of hemoglobin S/E during pregnancy.
  • Favorable pregnancy outcomes are suggested for homozygous hemoglobin E or hemoglobin E trait.
  • Further research is warranted to fully understand the risks and optimal management of S/E hemoglobinopathy in pregnancy.