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Pregnancy complicated by hereditary spherocytosis
M C Maberry1, R A Mason, F G Cunningham
1Department of Obstetrics and Gynecology, University of Texas Southwestern Medical Center, Dallas.
Obstetrics and Gynecology
|May 1, 1992
Summary
Pregnancy in women with hereditary spherocytosis generally yields good perinatal outcomes. While anemia is a risk, maternal complications are infrequent, and blood volume expansion is comparable to normal pregnancies.
Area of Science:
- Hematology
- Obstetrics
- Genetics
Background:
- Hereditary spherocytosis (HS) involves inherited defects in erythrocyte membranes, leading to hemolysis and anemia.
- These red blood cell abnormalities can impact pregnancy health and outcomes.
Purpose of the Study:
- To evaluate maternal and perinatal outcomes in pregnancies of women with hereditary spherocytosis.
- To assess physiological changes like blood volume expansion and red cell survival during pregnancy in HS patients.
Main Methods:
- Retrospective analysis of 50 pregnancies in 23 women diagnosed with hereditary spherocytosis.
- Measurement of blood volume expansion using 51chromium-tagged erythrocytes in a subset of patients.
- Assessment of mean red cell survival during pregnancy.
Main Results:
- Maternal complications were infrequent, with anemia being the most notable exception.
- Perinatal outcomes were generally favorable across the studied pregnancies.
- Blood volume expansion in HS patients was similar to that of normal pregnant women.
- Mean red cell survival varied significantly (30-90 days), reflecting the disorder's heterogeneity.
Conclusions:
- Pregnancy is generally well-tolerated in women with hereditary spherocytosis.
- The study highlights the importance of managing anemia during pregnancy in these patients.
- Observed red cell survival rates underscore the variable nature of hereditary spherocytosis.
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