[Study on prethrombotic state in patients with paroxysmal nocturnal hemoglobinuria]

To explore the mechanism of thrombogenesis in patients with paroxysmal nocturnal hemoglobinuria (PNH).

Thirteen parameters concerning endotheliocytes, platelets, blood coagulation, and fibrinolysis were measured in 21 PNH patients.

As compared with normal controls, plasma fibrin peptide A (FPA) levels were significantly higher, but antithrombin-III (AT-III) and protein C (PC) antigen levels lower in the PNH patients. Plasma tissue plasminogen activator (t-PA) activities were significantly lower while plasminogen activator inhibitor (PAI) activities markedly higher in the patients than those in the controls. The levels of the blood coagulation and fibrinolysis parameters changed more notably during the occurrence of thrombosis and hemoglobinuria episodes in the PNH patients. Platelet adhesion rates, platelet aggregation rates, beta-thromboglobulin (beta-TG), and granule membrane protein-140 (GMP-140) were within the normal ranges. Plasma vWF:Ag and endothelin-1 (ET-1) concentrations were normal in the patients as well.

Prethrombotic state is present in PNH patients, which may be caused by hypercoagulability and low fibrinolytic activity.