Acute splenic sequestration crises (ASSC) is one of the complications
of sickle cell disease (SCD) that can be life-threatening due to loss
of blood volume. Over a 5-year period, we have treated 19 patients ran
ging in age from 4 to 32 years with ASSC. There were 14 males and 5 fe
males; 17 had homozygous SCD and the other 2 had sickle thalassemia. T
wo patients presented with severe anemia and acute circulatory collaps
e; 1 of them developed residual weakness of his limbs and decreased vi
sual acuity. Nine patients underwent splenectomy after major episodes
of sequestration while the remaining 10 had recurrent minor episodes o
f sequestration. The clinical features and the role of splenectomy are
discussed.