NON-BENIGN SICKLE-CELL ANEMIA IN WESTERN SAUDI-ARABIA

Seventy-one Saudi and Yemeni Arabs with sickle cell anemia from western Saudi Arabia aged between 1.5 and 42 yr were studied. The mean steady state Hb concentration of 8.1 g/dl was lower than that of 10.7 g/dl reported previously for sickle cell anemia in eastern Saudi Arabia. The patients were divided into an SSLF group with fetal Hb (HbF) of 10.0% or below (44 patients) and an SSHF group having HbF above 10.0% (27 patients). No significant differences were found in the Hb concentrations, hematological indices and incidences of bone changes of the 2 groups. SSLF patients were significantly more prone to infections (P < 0.01), however. Also, there was an overall high incidence of hepatomegaly (69.0%) and splenomegaly (54.9%) and hepatomegaly was significantly more common in the SSLF group (P < 0.02). Many of the patients, even with HbF levels over 10.0%, did not follow a benign course and suffered from severe anemia, infections of the respiratory and urinary tracts, bone pains and infarcts, or bossing of the skull. Rarer complications included hepatic crisis, chest syndrome, retinal hemorrhage, epistaxis and hemiplegia. Saudi Arabian sickle cell anemias, even in patients with raised Hb F levels, may be as clinically severe as in African patients.