Fibrinogen degradation products in patients with the Quebec platelet disorder

The Quebec platelet disorder is a serious bleeding disorder associated with proteolysis of alpha-granular proteins, including fibrinogen. We evaluated fibrinogen degradation product (FDP) assays as screening tests for this disorder. Patients with the Quebec platelet disorder (13/13) had elevated serum FDPs (P < 0.01) due to secreted degraded platelet fibrinogen, but normal plasma FDPs and D-dimers. Unrelated controls with bleeding disorders (32/34) had undetectable FDPs, and controls with FDPs due to disseminated intravascular coagulation (DIG) and other illnesses (11/11) had elevated FDPs in all assays (P < 0.01). Immunoblot analyses indicated plasma fibrinogen was normal in the Quebec patients and platelet FDPs were found in their platelet lysates, releasates and serum samples. Their platelet FDPs were not altered by treatment with fibrinolytic inhibitors and were different from the FDPs in DIC and in plasmin-digested fibrinogen. Tests for serum FDPs may provide a simple rapid way to screen for the Quebec platelet disorder.