paroxysmal nocturnal haemoglobinuria
- paroxysmal nocturnal haemoglobinuria
a type of acquired haemolytic anaemia that results from an abnormality of the red blood cell membrane. It is due to a defect in the formation of glycosylphosphatidylinositol (GPI), whose role is to anchor proteins to the lipid framework of the membrane. This leads to increased complement-mediated destruction of red blood cells, which results in the release of haemoglobin in the circulation and then in the urine, giving the latter a reddish colour. Some patients may develop blood clots.
The new mediacal dictionary.
2014.
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paroxysmal nocturnal haemoglobinuria — Disease in which there is haemolysis by complement as a result of deficiency in decay accelerating factor … Dictionary of molecular biology
Paroxysmal nocturnal hemoglobinuria — Classification and external resources ICD 10 D59.5 ICD 9 283.2 … Wikipedia
PIGA — Phosphatidylinositol glycan anchor biosynthesis, class A (paroxysmal nocturnal hemoglobinuria), also known as PIGA, is a human gene.cite web | title = Entrez Gene: PIGA phosphatidylinositol glycan anchor biosynthesis, class A (paroxysmal… … Wikipedia
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decay accelerating factor — Plasma protein that regulates complement cascade by blocking the formation of the C3bBb complex (the C3 convertase of the alternate pathway). Widely distributed in tissues but deficient in paroxysmal nocturnal haemoglobinuria … Dictionary of molecular biology
P antigen — Antigenic determinant on the surface of human red blood cells to which the Donath Landsteiner antibody reacts. This antibody binds in the cold (a cold IgG ), but elutes from red cells at 37°C, is particularly associated with tertiary syphylis,… … Dictionary of molecular biology
methaemalbumin — methaemalbumin, methaemoglobin, methaemoglobinaemia, methaemoglobinuria chiefly Brit var of METHEMALBUMIN, METHEMOGLOBIN, METHEMOGLOBINEMIA, METHEMOGLOBINURIA * * * n. a chemical complex of the pigment portion of haemoglobin ( … Medical dictionary
methaemalbumin — n. a chemical complex of the pigment portion of haemoglobin (haem) with the plasma protein albumin. It is formed in the blood in such conditions as blackwater fever or paroxysmal nocturnal haemoglobinuria, in which red blood cells are destroyed… … The new mediacal dictionary
PNH — see paroxysmal nocturnal haemoglobinuria … The new mediacal dictionary
