Number of mutations and median phenotype in 150 index cases with type 1 VWD
| Multimer pattern . | No. of ICs per group . | No. of mutations per IC . | No. of mutations per group . | FVIII:C,† IU/dL . | VWF:RCo,† IU/dL . | VWF:Ag,† IU/dL . | VWF:CB†, IU/dL . | VWF:RCo/VWF:Ag,† . | % blood group O . | Bleeding score† . |
|---|---|---|---|---|---|---|---|---|---|---|
| Group 1* | ||||||||||
| AbM | 57 | 0, 1, or > 1 | 65 | 34 | 10 | 19 | 19 | 0.53 | 60 | 10.0 |
| AbM | 54 | 1 or > 1 | 65 | 33 | 10 | 19 | 17 | 0.53 | 61 | 10.0 |
| AbM | 43 | 1 | 43 | 33 | 10 | 20 | 19 | 0.50 | 61 | 10.0 |
| AbM | 11 | > 1 | 22 | 34 | 11 | 13 | 15 | 0.85 | 64 | 12.0 |
| AbM | 3 | 0 | 0 | 39 | 28 | 36 | 20 | 0.78 | 33 | 6.0 |
| Groups 2+3 | ||||||||||
| NM | 93 | 0, 1, or > 1 | 59 | 70 | 45 | 47 | 54 | 0.96 | 67 | 8.0 |
| Group 2 | ||||||||||
| NM | 51 | 1 or > 1 | 59 | 66 | 42 | 45 | 48 | 0.93 | 60 | 8.0 |
| NM | 44 | 1 | 44 | 67 | 43 | 45 | 49 | 0.96 | 61 | 7.5 |
| NM | 7 | > 1 | 15 | 30 | 38 | 47 | 38 | 0.81 | 71 | 10.0 |
| Group 3 | ||||||||||
| NM | 42 | 0 | 0 | 77 | 51 | 49 | 57 | 1.04 | 76 | 8.0 |
| All | ||||||||||
| AbM or NM | 150 | 0, 1, or > 1 | 124 | 58 | 35 | 38 | 39 | 0.92 | 65 | 9.0 |
| Multimer pattern . | No. of ICs per group . | No. of mutations per IC . | No. of mutations per group . | FVIII:C,† IU/dL . | VWF:RCo,† IU/dL . | VWF:Ag,† IU/dL . | VWF:CB†, IU/dL . | VWF:RCo/VWF:Ag,† . | % blood group O . | Bleeding score† . |
|---|---|---|---|---|---|---|---|---|---|---|
| Group 1* | ||||||||||
| AbM | 57 | 0, 1, or > 1 | 65 | 34 | 10 | 19 | 19 | 0.53 | 60 | 10.0 |
| AbM | 54 | 1 or > 1 | 65 | 33 | 10 | 19 | 17 | 0.53 | 61 | 10.0 |
| AbM | 43 | 1 | 43 | 33 | 10 | 20 | 19 | 0.50 | 61 | 10.0 |
| AbM | 11 | > 1 | 22 | 34 | 11 | 13 | 15 | 0.85 | 64 | 12.0 |
| AbM | 3 | 0 | 0 | 39 | 28 | 36 | 20 | 0.78 | 33 | 6.0 |
| Groups 2+3 | ||||||||||
| NM | 93 | 0, 1, or > 1 | 59 | 70 | 45 | 47 | 54 | 0.96 | 67 | 8.0 |
| Group 2 | ||||||||||
| NM | 51 | 1 or > 1 | 59 | 66 | 42 | 45 | 48 | 0.93 | 60 | 8.0 |
| NM | 44 | 1 | 44 | 67 | 43 | 45 | 49 | 0.96 | 61 | 7.5 |
| NM | 7 | > 1 | 15 | 30 | 38 | 47 | 38 | 0.81 | 71 | 10.0 |
| Group 3 | ||||||||||
| NM | 42 | 0 | 0 | 77 | 51 | 49 | 57 | 1.04 | 76 | 8.0 |
| All | ||||||||||
| AbM or NM | 150 | 0, 1, or > 1 | 124 | 58 | 35 | 38 | 39 | 0.92 | 65 | 9.0 |
P values using the Mann-Whitney test are as follows:
VWF:RCo group 1 versus 2 and group 1 versus 3, P < .001; group 2 versus 3, P = .033.
VWF:Ag group 1 versus 2 and group 1 versus 3, P < .001; group 2 versus 3, P = .127.
BS group 1 versus 2, P = .252; group 1 versus 3, P = .086; group 2 versus 3, P = .586.
The groups represent categories of ICs: 1, abnormal multimers (AbM); 2, normal multimers (NM) with a mutation; 3, normal multimers (NM) without a mutation.
Phenotypic data are indicated as the median.