Characteristics of Sickle Cell Anemia in Yemen

Abstract

We studied 136 males and 105 females with sickle cell anemia to determine the characteristics of the disease in Yemen. Their mean age [± SD (standard deviation)] was 12.8 ± 9.5 years (range: 9 months-40 years). Taiz, Hudaydah and Hajjah governorates, in the South-Central and the Northwestern provinces, showed the highest prevalence. Eighty percent of the patients had family history of the disease, 73.0% patients had history of parental consanguinity and 20.7% of death of relative(s) due to the disease; 5.4% patients were older than 30 years of age. Pain, jaundice and infection were the most frequent features. Splenomegaly, cholelithiasis, osteomyelitis, acute chest syndrome (ACS), osteonecrosis and stroke occurred in 12.0, 9.5, 8.7, 6.6, 6.6 and 2.9%, respectively. Priapism and leg ulcers were rare. The mean laboratory values (obtained in the steady state) were: hemoglobin (Hb) 7.9 g/dL, WBC 14.08 × 10⁹/L, platelet 460 × 10⁹/L, reticulocytes 14.5%, lactate dehydrogenase (LDH) 597 U/L, Hb F (α2γ2) 16.69%, Hb S [β6(A3)Glu→Val, GAG>GTG] 77.31% and Hb A₂ (α2δ2) 1.47%, respectively. There was no significant difference between South-Central and Northwestern provinces regarding clinical events and hematological parameters.

• Sickle cell anemia
• Yemen
• Malarial areas
• Consanguineous marriage
• Severe form
• High Hb F