What is the pathophysiology of thrombophilia in 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

The pathophysiology of thrombophilia in PNH is not fully understood, but the increased incidence during hemolytic episodes suggest a direct relationship with the hemolytic process. Increased procoagulant and fibrinolytic activity, suggesting increased fibrin generation and turnover, increased plasma levels of urokinase-type plasminogen activator and platelets deficient in GPI-linked proteins activated by complement, have been implicated. However, none of these identified platelet and coagulation abnormalities can fully explain the hypercoagulable state in PNH.


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