RED-CELL GENETIC-DISORDERS AND PLASMA-LIPIDS

To study the plasma lipid levels in patients with red cell genetic disorders, we investigated 400 normal individuals, 100 sickle cell disease (SCD) patients, 220 sickle cell heterozygotes (Hb AS), and 100 individuals suffering from glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, Whole blood samples were used for the determination of haematological parameters and red cell indices, and plasma was used for the estimation of plasma lipids using Autoanalyser American Monitor 'Parallel'. Haemoglobin types in the haemolysates were separated by electrophoresis and G-6-PD activity was determined using kits from Boehringer Mannheim GmbH. The results from males and females were analysed separately. In each group cholesterol levels were slightly higher in the male population, while the reverse was true for triglyceride. Sickle cell disease patients had significantly (P < 0.05) lower cholesterol level compared to the normal individuals. In the Hb AS and Ga-PD deficient groups no significant differences were encountered. Multiple regression analysis between cholesterol and haematological parameters showed a statistically significant positive correlation (P<0.01) between plasma cholesterol and total haemoglobin in each group, particularly in the sickle cell disease patients. The results suggest that increased utilization or decreased production may account for the lower cholesterol level in severely anaemic patients, particularly those with sickle cell anaemia.