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paroxysmal nocturnal haemoglobinuria


  • sometimes referred to as Marchiafava-Micheli syndrome, is a rare, acquired, potentially life-threatening disease of the blood characterised by complement-induced hemolytic anaemia, red urine (due to the appearance of haemoglobin in the urine) and thrombosis (in 40% of patients during their life).
  • the intravascular hemolysis tends to occur mainly at night - perhaps due to mild hypoxic acidosis of sleep, although it may be just because morning urine is more concentrated and it appears more red.
  • the first description of paroxysmal hemoglobinuria was by the German physician Paul Strübing (Greifswald, 1852-1915) in 1882.
  • it may develop on its own “primary”, or it may be secondary to bone marrow disorders such as aplastic anaemia.

clinical features

  • red urine in the morning (“nocturnal” haemoglobinuria)
  • haemolytic anaemia
    • episodes of haemolysis results in intravascular binding of nitric oxide and causes oesophageal spasm, erectile dysfunction, and abdominal pain, and the chronic depletion of nitric oxide is suspected in potentially causing pulmonary hypertension.
  • venous thrombosis tendency which increases risk of deep venous thrombosis (DVT), and thrombosis at unusual sites such as hepatic vein, portal vein, mesenteric veins, cerebral veins and cutaneous veins.
pnhb.txt · Last modified: 2010/01/18 04:23 by

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