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Association between Oxidative Stress, Genetic Factors, and Clinical Severity in Children with Sickle Cell Anemia

Céline Renoux1, Philippe Joly1, Camille Faes2

  • 1Inter-University Laboratory of Human Movement Biology (LIBM) EA7424, Team Vascular Biology and Red Blood Cell Team, University Claude Bernard Lyon, Villeurbanne, France; Laboratory of Excellence on Red Blood Cell (Labex GR-Ex), PRES Sorbonne, Paris, France; Department of Biochemistry on Red Blood Cell Disease, Biologie Est Center, Hospices Civils de Lyon, Lyon, France.

The Journal of Pediatrics
|February 17, 2018
PubMed

Insights

In sickle cell anemia, alpha-thalassemia may reduce oxidative stress, but oxidative stress levels did not correlate with vaso-occlusive events. This suggests alpha-thalassemia offers protection against certain sickle cell complications.

Area of Science:

  • Hematology
  • Genetics
  • Biochemistry

Background:

  • Sickle cell anemia is a genetic blood disorder characterized by abnormal hemoglobin.
  • Oxidative stress and genetic modifiers play roles in sickle cell disease (SCD) pathophysiology.
  • Understanding these factors is crucial for managing SCD complications.

Purpose of the Study:

  • To examine the relationship between SCD genetic modifiers (alpha-thalassemia, G6PD deficiency, beta-globin haplotypes) and oxidative stress levels.
  • To determine if oxidative stress correlates with the frequency of vaso-occlusive events in children with SCD.
  • To identify genetic factors that modulate oxidative stress in SCD.

Main Methods:

  • A 2-year study involving 62 children with sickle cell anemia and 12 healthy controls.
  • Measurement of oxidative/nitrosative stress markers, biological variables, and genetic modulators.
  • Tracking of vaso-occlusive crisis events requiring emergency admission.

Main Results:

  • Patients with sickle cell anemia exhibited higher oxidative and nitrosative stress than controls.
  • Alpha-thalassemia presence modulated oxidative stress levels in SCD patients.
  • Neither glucose-6-phosphate dehydrogenase deficiency nor beta-globin haplotypes affected oxidative stress levels.
  • The rate of vaso-occlusive crisis events was not associated with the level of oxidative stress.

Conclusions:

  • Alpha-thalassemia may mitigate oxidative stress in sickle cell anemia, potentially through reduced hemolysis.
  • This protective effect of alpha-thalassemia could explain its role in reducing hemolysis-related SCD complications.
  • Oxidative stress levels are not directly predictive of vaso-occlusive event frequency in this cohort.
Abstract

Keywords:
alpha thalassemiaoxidative stresssickle cell disease

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