Concept information
Preferred term
nocturnal paroxystic anemia
Definition(s)
- Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system. This destructive process occurs due to the presence of defective surface protein DAF on the red blood cell, which normally functions to inhibit such immune reactions. Since the complement cascade attacks the red blood cells within the blood vessels of the circulatory system, the red blood cell destruction (hemolysis) is considered an intravascular hemolytic anemia. Other key features of the disease, such as the high incidence of blood clot formation, are incompletely understood. (Wikipedia)
Broader concept(s)
In other languages
-
French
-
hémoglobinurie paroxystique nocturne
URI
http://data.loterre.fr/ark:/67375/VH8-W7L2XVNV-W
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