To study the plasma lipid levels in patients with red cell genetic dis
orders, we investigated 400 normal individuals, 100 sickle cell diseas
e (SCD) patients, 220 sickle cell heterozygotes (Hb AS), and 100 indiv
iduals suffering from glucose-6-phosphate dehydrogenase (G-6-PD) defic
iency, Whole blood samples were used for the determination of haematol
ogical parameters and red cell indices, and plasma was used for the es
timation of plasma lipids using Autoanalyser American Monitor 'Paralle
l'. Haemoglobin types in the haemolysates were separated by electropho
resis and G-6-PD activity was determined using kits from Boehringer Ma
nnheim GmbH. The results from males and females were analysed separate
ly. In each group cholesterol levels were slightly higher in the male
population, while the reverse was true for triglyceride. Sickle cell d
isease patients had significantly (P < 0.05) lower cholesterol level c
ompared to the normal individuals. In the Hb AS and Ga-PD deficient gr
oups no significant differences were encountered. Multiple regression
analysis between cholesterol and haematological parameters showed a st
atistically significant positive correlation (P<0.01) between plasma c
holesterol and total haemoglobin in each group, particularly in the si
ckle cell disease patients. The results suggest that increased utiliza
tion or decreased production may account for the lower cholesterol lev
el in severely anaemic patients, particularly those with sickle cell a
naemia.