Adverse clinical and obstetric outcomes among pregnant women with different sickle cell disease genotypes.

OBJECTIVE

To compare clinical and obstetric adverse events among pregnant women with sickle cell disease (SCD) according to genotype.

METHODS

The present cross-sectional study enrolled women aged 15-49 years with SCD and prior pregnancy attending a hematology center in Recife, Brazil, between September 1, 2015, and April 30, 2016. Associations between sickle cell genotype (HbSS, HbSC, Sβ-thalassemia) and adverse events were evaluated.

RESULTS

Overall, 89 women were included; 74 (83%) had HbSS genotype, 8 (9%) had HbSC genotype, and 7 (8%) had Sβ-thalassemia genotype. Fifty-three (60%) self-reported being of mixed race, and 27 (30%) self-reported they were black. Blood transfusion was observed more frequently among women with HbSS than among those with HbSC genotype (P=0.007). Postpartum adverse events were more frequent in the Sβ-thalassemia than in the HbSS group (P=0.030). Fetal intrauterine death occurred only among women with the HbSS genotype (11 [15%]). In the HbSS group, there was a higher frequency of blood transfusion (P=0.004) and lower rate of spontaneous abortion (P=0.001) among women with six or more consultations.

CONCLUSION

The HbSS genotype was associated with a higher frequency of blood transfusion. Sβ-thalassemia was associated with a higher frequency of postpartum adverse events. Prenatal care was associated with a lower rate of spontaneous abortion in the HbSS group.