What are the underlying pathophysiologic events of paroxysmal nocturnal hemoglobinuria (PNH)?

Updated: May 20, 2021
  • Author: Emmanuel C Besa, MD; Chief Editor: Sara J Grethlein, MD, FACP  more...
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Answer

PNH has been referred to as "the great impersonator" because of the variety of symptoms observed during its initial manifestation and course. This variety reflects the contributions of the following three underlying pathophysiologic events [1, 2, 3, 4, 5] :

  • An acquired intracorpuscular hemolytic anemia due to the abnormal susceptibility of the RBC membrane to the hemolytic activity of complement

  • Thromboses in large vessels, such as hepatic, abdominal, cerebral, and subdermal veins

  • A deficiency in hematopoiesis that may be mild or severe, such as pancytopenia in an aplastic anemia state

The triad of hemolytic anemia, pancytopenia, and thrombosis makes PNH a unique clinical syndrome.


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