Friday, 8 October 2010

MRCP revision battle 27.6: Paroxysmal nocturnal haemoglobinuria

Paroxysmal nocturnal haemoglobinuria is a condition in which blood cells are sensitive to complement-mediated lysis due to an inherited lack of surface glucosylphosphatidylinositol (GPI)

Features of paroxysmal nocturnal haemoglobinuria include:
  • cola coloured/red urine in morning due to increase in RBC lysis overnight
  • intravascular haemolysis
  • pancytopenia
  • thrombosis

Diagnosis is by urinary haemosiderin or + Ham's test (rarely done now)

Treatment is anticoagulation, transfusions when needed and possibly the smart new monoclonal antibody called eculizimab.

Now on to the last battle and some pretty pictures...