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Related Experiment Videos

Intracellular Ca(2+)-containing vesicles in sickle cell disorders

M P Westerman1, E Puchulu, R A Schlegel

  • 1Hematology/Oncology Unit, Mount Sinai Hospital, Chicago, IL 60608.

The Journal of Laboratory and Clinical Medicine
|September 1, 1994
PubMed
Summary

Calcium (Ca2+) vesicles in red blood cells are more frequent in severe sickle cell disorders. This finding correlates with disease severity, offering insights into sickle cell disease progression.

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Area of Science:

  • Hematology
  • Cell Biology

Background:

  • Sickle cell disorders are characterized by abnormal red blood cells.
  • Calcium (Ca2+) dysregulation is implicated in red blood cell pathophysiology.
  • Vesicle formation within red blood cells is a potential indicator of cellular stress.

Purpose of the Study:

  • To quantify the frequency of Ca(2+)-containing vesicles in red blood cells across different sickle cell disorder types.
  • To investigate the association between vesicle frequency and the clinical/hematologic severity of sickle cell disorders.
  • To assess the impact of deoxygenation on vesicle-containing red blood cells.

Main Methods:

  • Red blood cells from 49 sickle cell disorder patients were analyzed.
  • Cells were stained with chlortetracycline, a fluorescent probe for Ca(2+).

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  • Vesicle-containing cells were identified and quantified using microscopy.

    • Main Results:

    • Highest vesicle frequencies were found in sickle cell (SS) disease, S beta zero, and SS(-alpha/alpha alpha) thalassemias.
    • Lower frequencies were observed in SS(-alpha/-alpha) thalassemia and SS disease (Saudi Arabia high Hb F).
    • Normal low frequencies were seen in sickle cell-hereditary persistence of fetal hemoglobin and sickle trait.
    • Deoxygenation increased vesicle-containing cells proportionally to their oxygenated frequency.

    Conclusions:

    • The frequency of Ca(2+)-containing vesicles in sickle red blood cells is linked to the clinical and hematologic severity of the disorder.
    • Vesicle presence may serve as a biomarker for sickle cell disease severity.
    • These findings contribute to understanding the cellular mechanisms underlying sickle cell pathophysiology.