Does G gamma/A gamma ratio and Hb F level influence the severity of sickle cell anaemia.
Sickle cell anaemia (SCA) exhibits significant variations in clinical presentation in different populations for which several genetic factors including SCA-associated alpha- and beta-thalassaemias, G-6-PD deficiency and elevated Hb F level have been implicated as possible ameliorating factors. Saudi Arabia is unique in that mild and severe forms of the disease occur at a high frequency. We investigated the G gamma/A gamma ratio and Hb F level and correlated these values with the severity of SCA. The results showed that Hb F level varies significantly in both groups of patients with no evident correlation with the mild clinical manifestations. However, G gamma/A gamma ratio correlated significantly with the disease severity where a high ratio was observed in patients with the mild and a low ratio in patients with the severe disease. The results are evaluated and discussed in the light of correlation studies and regression analysis.
el-Hazmi
M A
MA
College of Medicine & King Khalid Hospital, King Saud University, Riyadh, Saudi Arabia.
Bahakim
H M
HM
Warsy
A S
AS
al-Momen
A
A
al-Wazzan
A
A
al-Fawwaz
I
I
Huraib
S
S
Harakati
M
M
D000755:Anemia, Sickle Cell / Q000378:metabolism* / Q000503:physiopathology*; D005319:Fetal Hemoglobin / Q000378:metabolism*; D005914:Globins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D012044:Regression Analysis
D005914:Globins; D005319:Fetal Hemoglobin