Abstract:The complement and coagulation systems are closely integrated with each influencing the activity of platelet in paroxysmal nocturnal hemoglobinuria(PNH).Thromboembolism is the most common cause of mortality and morbidity in patients with PNH.When thrombosis in an unusual site,or with evidence of hemolysis,or any cytopenia,testing for PNH by flow cytometry should be done.Activated platelets,release of arginase resulting in NO depletion,release of procoagulant red cell microparticles are related with thrombosis.Low-molecular-weight heparin,and continuing anticoagulation with the vitamin K antagonists is generally recommended.Allogeneic bone marrow transplant has been previously considered but could not improve the survival rate.Eculizumab treatment resulted in a reduction in the thromboembolism events effectively.