DOES G-GAMMA A-GAMMA RATIO AND HB F-LEVEL INFLUENCE THE SEVERITY OF SICKLE-CELL-ANEMIA/

Sickle cell anaemia (SCA) exhibits significant variations in clinical presentation in different populations for which several genetic factor s including SCA-associated alpha- and beta-thalassaemias, G-6-PD defic iency and elevated Hb F level have been implicated as possible amelior ating factors. Saudi Arabia is unique in that mild and severe forms of the disease occur at a high frequency. We investigated the Ggamma/Aga mma ratio and Hb F level and correlated these values with the severity of SCA. The results showed that Hb F level varies significantly in bo th groups of patients with no evident correlation with the mild clinic al manifestations. However, Ggamma/Agamma ratio correlated significant ly with the disease severity where a high ratio was observed in patien ts with the mild and a low ratio in patients with the severe disease. The results are evaluated and discussed in the light of correlation st udies and regression analysis.