Pregnancy in sickle cell disease: maternal and fetal outcomes in a population receiving prophylactic partial exchange transfusions.

OBJECTIVE To describe pregnancy outcomes for pregnant women with sickle cell disease (SCD) receiving prophylactic transfusions. STUDY DESIGN This retrospective case-control study compared pregnancy outcomes among women with SCD receiving prophylactic transfusions and women without any hemoglobinopathy, matched for ethnicity, parity, age and hospital. RESULTS The study included two groups of pregnancies: 128 in women with SCD (95 with SS phenotype and 33 with SC) and 128 in women with AA phenotype. No woman died. Two perinatal deaths (2.1%) and five alloimmunizations (5.3%) occurred, all in the SS group. Compared with the control group, HbSS disease was more often associated with pre-eclampsia (9.4% versus 2.3%, p=.03), preterm delivery (15.8% versus 6.2%, p=.01), birth weight <10th percentile (13.7% versus 3.9%, p=.008) and caesarean delivery (73.6% versus 26.4%, p<.01). CONCLUSION Despite prophylactic blood transfusions, SCD remains a severe complicating factor in pregnancy. The policy of systematic transfusions should be analyzed in a sufficiently large randomized trial.