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P Connes1, E Nader1

  • 1Laboratoire LIBM EA7424, équipe « biologie vasculaire et du globule rouge », faculté de médecine Laennec, université Lyon 1, France; Laboratoire d'excellence sur le globule rouge (GR-Ex), Paris, France.

La Revue De Medecine Interne
|December 4, 2023
PubMed
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Sickle cell anemia involves abnormal hemoglobin (HbS) polymerizing and causing red blood cell sickling. This leads to anemia, pain, and organ damage, highlighting key therapeutic targets for new treatments.

Area of Science:

  • Hematology
  • Genetics
  • Pathophysiology

Context:

  • Sickle cell anemia is a genetic blood disorder characterized by abnormal hemoglobin (HbS).
  • HbS polymerization under deoxygenated conditions causes red blood cell sickling, leading to various complications.
  • The disease exhibits a wide range of clinical manifestations due to altered red blood cell properties.

Purpose:

  • To elucidate the pathophysiology of sickle cell anemia.
  • To explore factors influencing HbS polymerization and red blood cell rheology.
  • To identify molecular and cellular alterations contributing to vascular complications and therapeutic targets.

Summary:

  • Abnormal hemoglobin (HbS) polymerizes, causing red blood cell sickling, rigidity, and fragility.
  • Sickled red blood cells lead to chronic hemolytic anemia, vaso-occlusive crises, and multi-organ vascular complications.
Keywords:
Blood rheologyDrépanocytoseFonction vasculaireGlobule rougeHémorhéologieRed blood cellSickle cell diseaseVascular function

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  • Factors like hydration, oxygen affinity, rheology, inflammation, and cell adhesion influence disease severity and progression.

    • Impact:

    • Understanding these mechanisms identifies critical therapeutic targets for novel sickle cell anemia treatments.
    • This research contributes to developing strategies to mitigate chronic hemolytic anemia and vascular complications.
    • The findings pave the way for interventions addressing red blood cell senescence, microparticle release, and endothelial adhesion.