paroxysmal nocturnal haemoglobinuria

paroxysmal nocturnal haemoglobinuria

A rare disease featuring episodic HAEMOLYSIS that occurs during the night when a decline in the pH of the blood triggers activation of complement components. The release of haemoglobin into the blood causes the morning urine to be black. The condition also features thrombosis and this may involve the hepatic vein causing the BUDD-CHIARI SYNDROME.