Sickle cell disease

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[edit] Discussion

  • Sickle cell disease is a chronic, hemolytic anemia, due to a single gene mutation, which conveys some resistance to malarial infection. During hypoxic crises, red blood cells alter shape, forming “sickles.” These altered RBCs occlude capillary beds, causing infarction. Structures which receive preferential blood supply are at highest risk.
  • Osseous Involvement: The proximal humerii, proximal femora, and vertebral bodies are often affected.
    • In the humerii, serpiginous sclerosis is characteristic of infarction, while the femoral heads are susceptible to avascular necrosis.
    • Sickle cell occlusion affects the mid portions of the vertebral endplates with relative sparring of the outer portions. This causes stunting of the growth of the central vertebrae and “over-growth,” actually normal growth, of the perimeter of the endplate. The difference in growth rate manifests in the “H” or “Lincoln log” appearance of the vertebral bodies.

[edit] Imaging Findings

  • Cardiomegaly
  • Bone infarcts
  • Avascular necrosis
  • Gallstones or history of cholecystectomy
  • Calcified or infarcted spleen

[edit] Images

Patient #1: SCD patient with H shaped vertebrae

Patient #2: SCD patient with H shaped vertebrae

[edit] See Also

[edit] External Links

[edit] References