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Pathology of sickle cell disease

Janet I Malowany1, Jagdish Butany

  • 1Department of Pathology and Laboratory Medicine, Toronto General Hospital/University Health Network, Toronto, Ontario, Canada.

Seminars in Diagnostic Pathology
|March 1, 2012
PubMed

Insights

Sickle cell disease (SCD) causes red blood cell sickling, leading to organ damage and premature death. This review examines SCD pathology, focusing on the causes of sudden death in affected individuals.

Area of Science:

  • Hematology
  • Genetics
  • Pathophysiology

Background:

  • Sickle cell disease (SCD) is an inherited hemolytic anemia with severe clinical outcomes.
  • Intravascular sickling of red blood cells causes multiorgan dysfunction in SCD patients.
  • Despite advancements in care, premature mortality remains a significant issue in SCD management.

Purpose of the Study:

  • To review the spectrum of pathology in sickle cell disease.
  • To emphasize the pathogenesis of sudden death in SCD.
  • To highlight the association between SCD and malaria resistance.

Main Methods:

  • Literature review of SCD pathophysiology.
  • Analysis of clinical consequences and mortality data.
  • Exploration of the genetic link between SCD and malaria.

Main Results:

  • SCD leads to widespread organ damage due to red blood cell sickling.
  • Sudden death is a recognized complication, with specific pathogenetic mechanisms.
  • Individuals with SCD exhibit a milder form of malaria, suggesting a survival advantage.

Conclusions:

  • Understanding SCD pathology is crucial for improving patient outcomes.
  • Further research into the pathogenesis of sudden death is warranted.
  • The protective effect against malaria in SCD carriers offers insights into evolutionary adaptation.

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