Paroxysmal nocturnal hemoglobinuria
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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening condition characterized by the destruction of red blood cells by the complement portion of the immune system.
This destructive process is a result of a defect in the formation of surface proteins on the red blood cell, which normally functions to inhibit such immune reactions. Common symptoms of PNH are fatigue due to anemia, abdominal pain, and thrombosis, which is the major cause of death. There can also be jaundice from the accumulation of bilirubin, and characteristic dark-colored urine.
The management of PNH involves drugs like eculizumab, a monoclonal antibody that blocks the activity of the complement system, anticoagulation therapy in case of thrombosis, blood transfusion, and supportive management as needed.