Paroxysmal nocturnal hemoglobinuria

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[edit] Discussion of Paroxysmal nocturnal hemoglobinuria

  • Paroxysmal nocturnal hemoglobinuria is an acquired disorder of hematopoietic stem cells that results in an abnormal predisposition to complement-mediated hemolysis.
  • Clinically, paroxysmal nocturnal hemoglobinuria results in a variable mix of acute and chronic hemolysis (nocturnal hemolysis is well recognized).
  • Acute episodes may be precipitated by infection, transfusion, exercise, drugs, immunization, surgery, or iodinated intravenous contrast medium.
  • Complications of paroxysmal nocturnal hemoglobinuria include:

[edit] Imaging Findings of Paroxysmal nocturnal hemoglobinuria

[edit] MRI

  • Renal cortical signal intensity loss (hemosiderin accumulates in the renal cortex when intravascular hemolysis results in the direct release of hemoglobin into the plasma).
  • Venous thrombosis.
  • Liver and spleen are usually of normal signal intensity in paroxysmal nocturnal hemoglobinuria, unless repeated transfusions have resulted in hepatic and splenic signal intensity loss owing to transfusional siderosis.

[edit] Images of Paroxysmal nocturnal hemoglobinuria

Patient #1: MR images demonstrate paroxysmal nocturnal hemoglobinuria

[edit] External Links

[edit] References for Paroxysmal nocturnal hemoglobinuria