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Red cell genetic disorders and plasma lipids

M A el-Hazmi1, A S Warsy, A al-Swailem

  • 1College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Journal of Tropical Pediatrics
|August 1, 1995
PubMed
Summary

Sickle cell disease patients exhibit significantly lower cholesterol levels compared to healthy individuals. Plasma cholesterol positively correlates with total hemoglobin, suggesting altered lipid metabolism in red cell disorders.

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Area of Science:

  • Hematology
  • Biochemistry
  • Genetics

Background:

  • Red cell genetic disorders, such as sickle cell disease (SCD) and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, can impact overall health.
  • Plasma lipid profiles are crucial indicators of cardiovascular health and metabolic status.

Purpose of the Study:

  • To investigate plasma lipid levels in individuals with SCD, sickle cell trait (Hb AS), and G-6-PD deficiency.
  • To explore the relationship between lipid levels and hematological parameters in these patient groups.

Main Methods:

  • Investigated 400 normal individuals, 100 SCD patients, 220 Hb AS heterozygotes, and 100 G-6-PD deficient individuals.
  • Determined hematological parameters, red cell indices, and plasma lipids using Autoanalyser.
  • Separated hemoglobin types via electrophoresis and measured G-6-PD activity.

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Main Results:

  • Sickle cell disease patients showed significantly lower cholesterol levels (P < 0.05) than normal individuals.
  • Plasma cholesterol demonstrated a significant positive correlation with total hemoglobin (P < 0.01) in all groups, especially in SCD patients.
  • No significant differences in lipid levels were found in Hb AS and G-6-PD deficient groups compared to controls.

Conclusions:

  • Severely anemic patients, particularly those with sickle cell anemia, may have reduced cholesterol levels due to increased utilization or decreased production.
  • These findings highlight a potential link between red cell disorders and altered lipid metabolism.