Table 1.

Demographic and laboratory data of all patients with MBDs and healthy controls

All patients, n (%) or median [IQR] (N = 620)Controls, n (%) or median [IQR] (n = 100)P
Age, y 40 [28-53] 38 [28-50] .386 
Female 505 (81.5) 81 (81.0) .914 
Blood group O 314 (51.1) 31 (31.0) <.001 
Positive family history 232 (37.4) na na 
BMI, kg/m2 23.1 [21.0-26.2] 22.2 [20.7-25.2] .044 
Hemoglobin, g/dL 13.6 [12.8-14.4] 13.7 [13-14.5] .196 
Platelet count, ×109/L 225 [209-284] 264 [224-294] .095 
aPTT-STA, s 35.9 [33.7-38.9] 35.1 [33.3-36.4] <.001 
Prothrombin time, % 95 [88-104] 100 [92-109] <.001 
Fibrinogen, mg/dL 306.5 [266.0-353.8] 290.0 [244.0-340.3] .070 
FVIII, % 120.0 [96.0-153.0] 131.0 [105.5-157.8] .058 
FIX, % 104.0 [89.0-120.0] 100.0 [89.8-114.0] .213 
VWF:Ag, % 94.0 [72.0-117.0] 104.0 [93.0-135.0] <.001 
VWF:RCo, % 78.0 [63.0-116.5] 93.0 [71.0-127.3] .002 
Vicenza Bleeding Score 5 [4-8] 0 [0-0] <.001 
ISTH BAT* 6 [4-9] 0 [0-0] <.001 
All patients, n (%) or median [IQR] (N = 620)Controls, n (%) or median [IQR] (n = 100)P
Age, y 40 [28-53] 38 [28-50] .386 
Female 505 (81.5) 81 (81.0) .914 
Blood group O 314 (51.1) 31 (31.0) <.001 
Positive family history 232 (37.4) na na 
BMI, kg/m2 23.1 [21.0-26.2] 22.2 [20.7-25.2] .044 
Hemoglobin, g/dL 13.6 [12.8-14.4] 13.7 [13-14.5] .196 
Platelet count, ×109/L 225 [209-284] 264 [224-294] .095 
aPTT-STA, s 35.9 [33.7-38.9] 35.1 [33.3-36.4] <.001 
Prothrombin time, % 95 [88-104] 100 [92-109] <.001 
Fibrinogen, mg/dL 306.5 [266.0-353.8] 290.0 [244.0-340.3] .070 
FVIII, % 120.0 [96.0-153.0] 131.0 [105.5-157.8] .058 
FIX, % 104.0 [89.0-120.0] 100.0 [89.8-114.0] .213 
VWF:Ag, % 94.0 [72.0-117.0] 104.0 [93.0-135.0] <.001 
VWF:RCo, % 78.0 [63.0-116.5] 93.0 [71.0-127.3] .002 
Vicenza Bleeding Score 5 [4-8] 0 [0-0] <.001 
ISTH BAT* 6 [4-9] 0 [0-0] <.001 

FIX, factor IX activity; FVIII, factor VIII activity; ISTH BAT, International Society on Thrombosis and Haemostasis Bleeding Assessment Tool; na, not available.

*

ISTH Bleeding Score available for 359 patients (57.9%).

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