Acute Chest Syndrome in Pregnant Women with Hemoglobin SC Disease

Acute chest syndrome (ACS) is a severe pulmonary complication in pregnant women with sickle cell disease (SCD). Between January 1998 and December 2002, ACS occurred in 4 of 286 pregnant women with SCD in Bahrain (1.2%), and it was associated with two maternal deaths. In the United States, 10 pregnant women (0.06%) were diagnosed with ACS out of 17,952 deliveries in women with SCD from 2000 to 2003. ACS has been attributed to pulmonary vasoocclusion and fat emboli originated from bone marrow infarctions with subsequent hypoventilation. Infectious etiology has also been considered. The pregnancy outcome for patients with hemoglobin SC disease has been reported to be good to optimal when compared to the outcome of those with homozygous hemoglobin S (Hb SS). Although most SC patients are mildly affected by clinical events, some of them develop severe complications. We report two cases of severe ACS in pregnant women with hemoglobin SC disease, including maternal and perinatal outcome.