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Related Experiment Videos

The reversibly sickled cell

A Zipursky1, D M Chachula, E J Brown

  • 1Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.

The American Journal of Pediatric Hematology/Oncology
|May 1, 1993
PubMed
Summary
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New techniques detect sickled cells in sickle cell disease (SCD). Higher irreversibly sickled cells (ISCs) and reversibly sickled cells (RSCs) correlate with disease severity, not crisis frequency, suggesting local stasis causes SCD complications.

Area of Science:

  • Hematology
  • Pediatrics
  • Vascular Biology

Background:

  • Sickle cell disease (SCD) involves red blood cell abnormalities.
  • Irreversibly sickled cells (ISCs) and reversibly sickled cells (RSCs) are key indicators.
  • Understanding ISC and RSC dynamics is crucial for managing SCD.

Purpose of the Study:

  • To develop and apply simple techniques for detecting ISCs and RSCs.
  • To quantify ISC and RSC levels in children with sickle cell disease (SS) and sickle cell C disease (SC).

Main Methods:

  • Developed simple detection techniques for ISCs and RSCs.
  • Analyzed blood samples from 100 children with SS and 20 with SC disease.

Main Results:

  • ISC and RSC counts were significantly higher in SS disease compared to SC disease.

Related Experiment Videos

  • Higher ISC and RSC numbers correlated directly with hemolytic process severity.
  • ISC and RSC numbers showed a negative relationship with vaso-occlusive crisis frequency.

    • Conclusions:

    • Sickle cell disease manifestations are likely due to local vascular stasis and subsequent sickling, not random lodging of sickled cells.
    • Vaso-occlusion is a critical factor in SCD pathogenesis.
    • The study provides insights into the pathophysiology of sickle cell disease.