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Hemolytic processes in paroxysmal nocturnal hemoglobinuria and its treatment: intravascular and extravascular hemolysis
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare and lethal disorder of the blood in which the body attacks and destroys red blood cells in massive amounts. This focus collection and short animation brings together important papers on the treatment of PNH patients and the associated risks caused by intravascular and extravascular hemolysis.
Anaemia, thrombosis and smooth muscle dystonias are manifestations of paroxysmal nocturnal haemoglobinuria, a rare but potentially life-threatening haematological disorder. Inhibition of complement activation is the treatment of choice; stem cell transplantation is recommended for patients who also develop severe bone marrow failure.