Hemorrhagic symptoms and bleeding risk in obligatory carriers of type 3 von Willebrand disease in southern Iran.

The objective of the present study was to compare old and new bleeding scores in patients with type-3 von Willebrand disease (vWD), obligatory carriers and normal controls, and to compare the ability of bleeding scores vs. clinical and laboratory data to predict bleeding after surgery. We identified 15 patients from 12 families who had type 3 vWD. Normal controls were matched to carriers by sex and age. Two physician-administered standardized questionnaires were used to evaluate old and new bleeding symptoms. Scores for old symptoms were the same in carriers and control participants (median score 0.00 vs. 0.00, P < 0.001), and patients with vWD had a significantly higher bleeding score than carriers (median 10.00 vs. 0.00, P < 0.001). Scores for new symptoms were higher in carriers than in control participants (median score -1.00 vs. -2.00, P < 0.001), and patients had a significantly higher bleeding score than carriers (median 14.00 vs. -1.00, P < 0.001). The clinical situations associated with increased bleeding risk (old symptoms) in patients with type 3 vWD compared to obligatory carriers were epistaxis [odds ratio (OR) = 175.5; 95% confidence interval (CI) 14.55-2116.69; P < 0.001], cutaneous symptoms (OR = 108; 95% CI 10.16-1147.39; P < 0.001) and hemarthrosis (OR = 19.5%; 95% CI 4.32-156.46; P < 0.001). The clinical situations associated with increased bleeding risk according to scores for new symptoms in patients with type 3 vWD compared to obligatory carriers were epistaxis (OR = 175.5; 95% CI 14.55-2116.69; P < 0.001), cutaneous symptoms (OR = 52; 95% CI 7.65-353.09; P < 0.001) and bleeding from minor wounds (OR = 74.25; 95% CI 7.43-741.118; P < 0.001). The three groups differed significantly in the severity of epistaxis and cutaneous bleeding according to scores for new and old symptoms. The new bleeding score was more reliable than the old bleeding score in predicting bleeding after invasive procedure.