Natural History of Sickle Cell Anemia in Saudi Arabs

Abstract

Saudi Arabs [270] with homozygous sickel cell anemia were studied using chart review, a register (since 1969) and home visiting in 42 cases. Average follow-up for the total group was 10 yr; 74% of those diagnosed by age 3 yr presented on screening or with merely anemia; 26% presented with illness, abnormal physical findings or pain. Compared with American or Jamaican blacks, serious complications occurred only 6% to 25% as frequently; leg ulcers did not occur at all; the mortality under age 15 yr was 10% as great; mean levels of blood Hb were higher (10 g/dl), reticulocyte count was lower (5% to 6%), and mean fetal Hb (HbF), which was inversely correlated with reticulocytes, was higher (22% to 26.8%). The high HbF is believed to account for the very mild clinical manifestations.