Congenital
factor VII (FVII) deficiency is characterized by genotypic variability and phenotypic heterogeneity. Traditional screening and factor assays are unable to reliably predict clinical
bleeding phenotype and guide haemorrhage prevention strategy. Global assays of coagulation and fibrinolysis may better characterize overall haemostatic balance and aid in haemorrhagic risk assessment. We evaluated the ability of novel global assays to better understand clinical
bleeding severity in congenital FVII deficiency. Subjects underwent central determination of
factor VII activity (
FVII:C) as well as clot formation and lysis (CloFAL) and simultaneous
thrombin and
plasmin generation (STP) global assay analysis. A
bleeding score was assigned to each subject through medical chart review. Global assay parameters were analysed with respect to
bleeding score and
FVII:C. Subgroup analyses were performed on paediatric subjects and subjects with FVII ≥ 1 IU dL(-1). CloFAL fibrinolytic index (FI2 ) inversely correlated with
FVII:C while CloFAL maximum amplitude (MA) and STP maximum velocity of
thrombin generation (VT max) varied directly with
FVII:C. CloFAL FI2 directly correlated with
bleeding score among subjects in both the total cohort and paediatric subcohort, but not among subjects with FVII ≥ 1 IU dL(-1) . Among subjects with FVII ≥ 1 IU dL(-1), STP time to maximum velocity of
thrombin generation and time to maximum velocity of
plasmin generation inversely correlated with
bleeding score. These preliminary findings suggest a novel potential link between a hyperfibrinolytic state in
bleeding severity and congenital FVII deficiency, an observation that should be further explored.