The thrombogram in rare inherited coagulation disorders: its relation to clinical XXXX .

@entity1 @entity548 @entity4059 @entity5630 @entity942
We investigated the relation between clotting factor concentration, the parameters of the thrombin generation curve (the thrombogram) and the severity of clinically observed @entity548 in @entity1 with @entity942 of prothrombin (n = 21), factor V (n = 22), factor VII (n = 22), factor X (n = 10), factor XI (n = 7) and factor XII (n = 6). The parameters used were: area under the curve (endogenous thrombin potential, ETP), peak concentration of thrombin attained and lag time before manifest formation. Peak height and ETP varied linearly with the concentration of prothrombin. For the other factors these parameters hyperbolically approached to the 100% limit with increasing clotting factor concentration. Half normal ETP was seen at about the following concentrations: prothrombin (50%), factor V (1%), factor VII (2%), factor X (5%) and factor XI (1%). As a rule, the peak height was some-what more sensitive to clotting factor decrease than the ETP was. In all the @entity1 with severe @entity548 symptoms the ETP was less than 20% of normal. Bleeding tendency was absent or mild in @entity1 with an ETP of 30% or higher. This value (except for prothrombin) is already obtained at concentrations of clotting factor of 1%-2%, which corroborates the clinical observation that a severe @entity548 tendency is only seen in severe clotting @entity4059 (less than 1%). The one exception was a @entity1 with @entity5630 and severe @entity548 , who showed a normal ETP value, albeit with a decreased peak height and a prolonged lag-time.
