Table 1. Baseline characteristics and BAT scores of included patients ( n = 156) .
Age (y) | Median (range) | 42.5 (18–76) |
---|---|---|
Sex | Female, n (%) | 120 (76.9) |
Classification a |
Congenital platelet defect,
n
(%)
Isolated thrombocytopenia ADP pathway defect Glanzmann thrombasthenia TxA2 pathway defect Dense granule deficiency Complex abnormalities b Bernard–Soulier syndrome Possible congenital platelet defect Acquired platelet defect Von Willebrand disease Unexplained bleeding tendency |
77 (48.4) 20 (12.8) 15 (9.6) 13 (8.3) 11 (7.1) 8 (5.1) 6 (3.8) 4 (2.6) 16 (10.3) 3 (1.9) 1 (0.6) 59 (37.8) |
ISTH-BAT score | Median (range) Score 0 (floor score), n (%) Score > 5 for women, n (%) Score > 3 for men, n (%) |
10 (0–26) 5 (3.2) 104 (86.7) 25 (69.4) |
Self-BAT score | Median (range) Score 0 (floor score), n (%) Score > 5 for women, n (%) Score > 3 for men, n (%) |
12 (0–33) 2 (1.3) 110 (91.7) 29 (80.6) |
Abbreviations: ADP, adenosine diphosphate; ISTH-BAT, International Society for Thrombosis and Hemostasis–bleeding assessment tool; self-BAT, self-administered ISTH-BAT; TxA2, Thromboxane A2.
Final diagnosis at the end of the “Thrombocytopathy in the Netherlands” study.
The patterns of platelet function defects did not support the diagnosis of one particular congenital platelet defect.