Characteristics of type 3 VWD patients with and without VWFpp
| Characteristic . | Type 3 VWD patients . | P . | |||
|---|---|---|---|---|---|
| Without VWFpp (n = 22) . | With VWFpp (n = 15) . | ||||
| No. . | % . | No. . | % . | ||
| Age, y | .636 | ||||
| Median | 22 | 35 | |||
| Range | 2-60 | 4-65 | |||
| Male sex | 9 | 41 | 7 | 47 | .729 |
| Blood group O | 8 | 36 | 12 | 80 | .009 |
| VWFpp, U/dL | <.001 | ||||
| Median | 0 | 72 | |||
| 25%-75% IQR | 0-0 | 37-94 | |||
| VWF:Ag, IU/dL | <.001 | ||||
| Median | 0 | 4 | |||
| 25%-75% IQR | 0-1 | 2-4 | |||
| VWF:Act, U/dL | .036 | ||||
| Median | 0 | 1 | |||
| 25%-75% IQR | 0-0 | 0-3 | |||
| FVIII:C, IU/dL | <.001 | ||||
| Median | 2 | 9 | |||
| 25%-75% IQR | 1-3 | 8-13 | |||
| Multimers | |||||
| Absent | 19 | 86 | 3 | 20 | <.001 |
| Abnormal | 3 | 14 | 12 | 80 | |
| BS | .025 | ||||
| Median | 19.5 | 14.0 | |||
| 25%-75% IQR | 11.3-23.8* | 7.0-17.0 | |||
| Characteristic . | Type 3 VWD patients . | P . | |||
|---|---|---|---|---|---|
| Without VWFpp (n = 22) . | With VWFpp (n = 15) . | ||||
| No. . | % . | No. . | % . | ||
| Age, y | .636 | ||||
| Median | 22 | 35 | |||
| Range | 2-60 | 4-65 | |||
| Male sex | 9 | 41 | 7 | 47 | .729 |
| Blood group O | 8 | 36 | 12 | 80 | .009 |
| VWFpp, U/dL | <.001 | ||||
| Median | 0 | 72 | |||
| 25%-75% IQR | 0-0 | 37-94 | |||
| VWF:Ag, IU/dL | <.001 | ||||
| Median | 0 | 4 | |||
| 25%-75% IQR | 0-1 | 2-4 | |||
| VWF:Act, U/dL | .036 | ||||
| Median | 0 | 1 | |||
| 25%-75% IQR | 0-0 | 0-3 | |||
| FVIII:C, IU/dL | <.001 | ||||
| Median | 2 | 9 | |||
| 25%-75% IQR | 1-3 | 8-13 | |||
| Multimers | |||||
| Absent | 19 | 86 | 3 | 20 | <.001 |
| Abnormal | 3 | 14 | 12 | 80 | |
| BS | .025 | ||||
| Median | 19.5 | 14.0 | |||
| 25%-75% IQR | 11.3-23.8* | 7.0-17.0 | |||
Historical data on mutations (total of 11 missing): for those without VWFpp, 10 of 12 were homozygous or compound heterozygous for null alleles; for those with VWFpp, 14 patients were genotyped, 10 of whom had a mutation associated with increased clearance. VWFpp, VWF:Ag, VWF:Act, and FVIII:C levels were measured centrally at time of inclusion in the study.
Total of 2 missing.