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Crisis in sickle cell trait.

A W Dudley1, C C Waddell

  • 1Department of Pathology, Veterans Administration Medical Center, Houston, TX 77030.

Human Pathology
|June 11, 1991
PubMed
Summary
This summary is machine-generated.

A patient with sickle cell trait (hemoglobin AS) experienced a severe sickle cell crisis, leading to renal failure, neurological symptoms, and cardiac arrest. Autopsy confirmed sickled cells in key organs, indicating a progressive crisis.

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Area of Science:

  • Nephrology
  • Hematology
  • Neurology

Background:

  • Sickle cell trait (hemoglobin AS) is generally considered benign, but rare cases of sickle cell disease-like complications can occur.
  • Understanding the pathophysiology of sickle cell crisis in AS patients is crucial for early diagnosis and management.

Observation:

  • A 34-year-old male with hemoglobin AS presented with acute renal failure, polydipsia, hypertension, and altered mental status including confusion and visual hallucinations.
  • Initial management included dialysis for electrolyte abnormalities, but blood samples showed hemolysis and hyperkalemia.
  • Electrocardiogram revealed peaked T waves, preceding cardiac arrest.

Findings:

  • Autopsy revealed extensive sickling of erythrocytes within the visual cortex and other symptomatic organs.

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  • Notably, most of the peripheral vascular tree contained normal erythrocytes, suggesting localized or progressive vaso-occlusion.
  • These pathological findings support a diagnosis of a true, progressive sickle cell crisis in a patient with sickle cell trait.

    • Implications:

    • This case highlights the potential for severe, life-threatening complications in individuals with sickle cell trait.
    • It underscores the importance of considering sickle cell crisis in the differential diagnosis of patients with hemoglobin AS presenting with multi-organ dysfunction.
    • Further research into the triggers and mechanisms of progressive sickle cell crisis in AS patients is warranted.