Table 1.

Demographic and clinical characteristics of patients with severe FV deficiency

Patient	Sex	Age, y	FV level, %	Coinherited thrombophilic defects	Clinical phenotype*
PD I	F	64	< 0.5		Moderate/severe (5)
PD II	F	44	< 0.5	170% FVIII	Severe (7)
PD III	F	35	0.6	131% PT (no F2 G20210A)	Asymptomatic (0†)
PD IV	F	27	< 0.5		Mild (2)
PD V	F	52	< 0.5		Severe (10†)
PD VI	M	28	< 0.5		Mild (1†)
PD VII	F	62	4.8		Mild (1)
PD VII-A	F	46	6.2		Mild (1)
GK 502	F	57	< 0.5		Mild
GK 505	F	56	< 0.5	53% PC	Unknown
GK 506	M	65	< 0.5		Unknown

PT indicates prothrombin; and PC, protein C.

^*Numbers in parentheses represent the bleeding score calculated according to Rodeghiero et al.²⁹

^†Prophylaxis (with plasma and/or antifibrinolytic agents) often given during risk situations after the diagnosis of severe FV deficiency was made.