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Updated: Jul 14, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Natural history of blood pressure in sickle cell disease pregnancy
Macy L Early1, Amy Luo2, Marissa Solow3,4
1Division of Hematology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Insights
Pregnant individuals with sickle cell disease (SCD) often have lower blood pressure than those without SCD. This may lead to underdiagnosis of hypertensive disorders of pregnancy (HDP) in this vulnerable population.
Area of Science:
- Obstetrics and Gynecology
- Hematology
- Maternal-Fetal Medicine
Background:
- Sickle cell disease (SCD) significantly impacts pregnancy outcomes.
- Hypertensive disorders of pregnancy (HDP) are a major concern for pregnant individuals with SCD.
- Understanding blood pressure patterns in SCD pregnancies is crucial for timely diagnosis and management.
Purpose of the Study:
- To compare blood pressure values and trajectories in singleton pregnancies with SCD against a non-SCD control group.
- To investigate potential differences in blood pressure and HDP rates across SCD genotypes (HbSS/HbSβ0 and HbSC/HbSβ+).
- To explore the relationship between blood pressure, SCD genotype, and HDP incidence.
Main Methods:
- Retrospective cohort study of 290 singleton pregnancies in individuals with SCD (1990-2021) at two academic centers.
- Collected demographic, SCD-related data, pregnancy outcomes, and serial systolic and diastolic blood pressure (SBP and DBP) measurements.
- Compared SCD cohort blood pressure data with a non-SCD pregnancy control dataset.
Main Results:
- Sixteen percent of pregnancies with SCD experienced a hypertensive disorder of pregnancy (HDP), with no significant difference by genotype.
- Mean SBP and DBP were consistently lower in the HbSS/HbSβ0 group compared to non-SCD controls.
- Blood pressure trajectories in HbSS/HbSβ0 pregnancies mirrored controls, while HbSC/HbSβ+ pregnancies showed a dip and plateau in the second and third trimesters, respectively.
Conclusions:
- Pregnant individuals with SCD generally exhibit lower blood pressure than their non-SCD counterparts.
- The lower blood pressure in SCD pregnancies, particularly HbSS/HbSβ0, may mask or lead to underdiagnosis of HDP.
- Further research is needed to refine diagnostic criteria for HDP in the context of SCD.
Abstract:
In this retrospective cohort study of singleton pregnancies in people with sickle cell disease (SCD) delivered at two academic centres between 1990 and 2021, we collected demographic and SCD-related data, pregnancy outcomes, and the highest systolic and diastolic blood pressure (SBP and DBP) at seven time periods. We compared blood pressure values and trajectories in the composite cohort and in each genotype group to control values in a non-SCD pregnancy dataset. There were 290 pregnancies among 197 patients with SCD. Sixteen per cent (n = 47) of pregnancies had a hypertensive disorder of pregnancy (HDP); the rates did not differ by genotype. The mean SBP and DBP were lower in the HbSS/HbSβ0 group than in the non-SCD control group at all timepoints. Mean SBP and DBP trajectories were similar between the HbSS/HbSβ0 group and non-SCD controls, whereas the mean SBP and DBP in the HbSC/HbSβ+ group decreased between the first and second trimesters and plateaued between the second and third trimesters. There were no differences in blood pressure trajectory by haemoglobin >/< 10 gm/dL or by chronic transfusion status. Overall, pregnant people with SCD have lower blood pressure than unaffected pregnant people, raising the possibility that HDP are underdiagnosed, particularly in people with HbSS/HbSβ0 .
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