Phenotypic data for affected members of the families investigated
| Family . | Patient . | Generation . | Bleeding time (min) . | FVIII:C (U/mL) . | vWF:Ag (U/mL) . | vWF:Ricof (U/mL) . | vWF:Ag Multimer . | |
|---|---|---|---|---|---|---|---|---|
| Plasma . | Platelet . | |||||||
| F1 | P1 | II:1 | 17 | 1.42 | 2.02 | 0.25 | Abnormal | Abnormal |
| F1 | P2 | III:1 | 17 | 0.64 | 1.16 | 0.33 | Abnormal | Abnormal |
| F1 | P3 | IV:1 | 19 | 0.58 | 0.89 | 0.30 | Abnormal | Abnormal |
| F1 | P4 | V:1 | Unavailable | 1.14 | 1.05 | 0.59 | Abnormal | Not done |
| F2 | P1 | I:1 | Unavailable | Unavailable | Unavailable | Unavailable | Abnormal | Not done |
| F2 | P2 | II:1 | >20 | 0.66 | 0.49 | 0.27 | Abnormal | Abnormal |
| F3 | P1 | — | >20 | 0.78 | 0.54 | 0.27 | Abnormal | Abnormal |
| Family . | Patient . | Generation . | Bleeding time (min) . | FVIII:C (U/mL) . | vWF:Ag (U/mL) . | vWF:Ricof (U/mL) . | vWF:Ag Multimer . | |
|---|---|---|---|---|---|---|---|---|
| Plasma . | Platelet . | |||||||
| F1 | P1 | II:1 | 17 | 1.42 | 2.02 | 0.25 | Abnormal | Abnormal |
| F1 | P2 | III:1 | 17 | 0.64 | 1.16 | 0.33 | Abnormal | Abnormal |
| F1 | P3 | IV:1 | 19 | 0.58 | 0.89 | 0.30 | Abnormal | Abnormal |
| F1 | P4 | V:1 | Unavailable | 1.14 | 1.05 | 0.59 | Abnormal | Not done |
| F2 | P1 | I:1 | Unavailable | Unavailable | Unavailable | Unavailable | Abnormal | Not done |
| F2 | P2 | II:1 | >20 | 0.66 | 0.49 | 0.27 | Abnormal | Abnormal |
| F3 | P1 | — | >20 | 0.78 | 0.54 | 0.27 | Abnormal | Abnormal |
C indicates coagulation; vWF, von Willebrand factor; Ag, antigen; Ricof, ristocetin cofactor.