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Sickle cell shape and structure: images and concepts (1840-1980)
Blood Cells
|January 1, 1982
Summary
Sickle cell disease involves abnormal hemoglobin causing red blood cells to adopt a sickle shape, leading to various health complications. Research details the discovery and understanding of this condition, including its genetic basis and diverse clinical presentations.
Area of Science:
- Hematology
- Genetics
- Pathology
Background:
- Early observations identified unique
- crescentic particles
- in animals and described sickle-shaped red blood cells in humans.
Observation:
- Sickling is linked to hemoglobin oxygenation states, initially noted as reversible and later as a result of asphyxia.
- Electron microscopy revealed rod-like hemoglobin structures within sickle cells.
- Irreversible sickle cells and diverse sickle cell shapes (drepanocytes, discodrepanocytes, etc.) were characterized.
Findings:
- The sickle cell trait represents a latent form of sickling.
- Sickling disorders exhibit heterogeneity, including associations with other hemolytic anemias and non-hemoglobin S causes.
- Myelin forms and agglutination of sickle cells are observed phenomena.
Implications:
- Understanding the morphology and pathophysiology of sickling is crucial for diagnosing and managing sickle cell disease.
- Further research into the molecular mechanisms of sickling can inform therapeutic strategies.
- Recognizing the heterogeneity of sickling disorders is essential for personalized patient care.