TABLE 2.
VWF mean values (IQR) | Non‐VWD (n = 1005) | VWD (n = 454) | Low VWF:RCo cohort (n = 82) | Low VWF:RCo + p.D1472H (n = 19) | Low VWF:RCo + p.D1472H FU (n = 15) | Low VWF:RCo ‐ p.D1472H FU (n = 22) |
---|---|---|---|---|---|---|
Local VWF:Ag IU/dl | 98 (78–124) | 50 (41–63) | 62 (55–66) | 63 (58–69) | 68 (59–74) | 67 (55–74) |
Local VWF:RCo IU/dl | 88 (66–114) | 42 (36–52) | 43 (40–48) | 44 (41–49) | 46 (39–58) | 57 (43–68) |
Local VWF:RCo/VWF:Ag | 0.9 (0.8–1.0) | 0.8 (0.7–1.0) | 0.7 (0.6–1.0) | 0.7 (0.6–0.8) | 0.7 (0.5–0.8) | 0.9 (0.8–1.0) |
BRI VWF:Ag IU/dl | 94 (74–117) | 50 (42–62) | 63 (53–69) | 63 (59–69) | 62 (54–70) | 62 (46–72) |
BRI VWF:GPIbM IU/dl | 110 (84–142) | 56 (46–75) | 69 (58–82) | 61 (52–72) | 65 (57–74) | 80 (55–112) |
BRI VWF:GPIbM/VWF:Ag | 1.2 (1.0–1.4) | 1.1 (1.0–1.4) | 1.1 (0.9–1.3) | 1.0 (0.9–1.0) | 1.1 (0.9–1.3) | 1.3 (1.1–1.6) |
Median ISTH‐BAT BS (range) | 3 (0–24) | 4 (0–34) | 3 (0–21) | 2 (0–21) | − | − |
Note: Mean and interquartile range (IQR) of local von Willebrand factor (VWF) antigen (VWF:Ag), VWF ristocetin cofactor activity (VWF:RCo), central (BRI) VWF antigen (VWF:Ag) and VWF glycoprotein‐IbM activity (VWF:GPIbM) and median ISTH‐BAT bleeding score (BS) in subjects without VWD (non‐VWD), with suspected VWD and those with a single low VWF:RCo with (+) and without (−) common p.D1472H variant at time of study entry and follow‐up (FU).