Hemostasis variables in the patients (boldface) and their relatives
| Proband . | Bleeding time (min) [< 6] . | FVIII:C (U/mL) [> 0.6] . | vWF:Ag (U/mL) [> 0.5] . | vWF:Ag (plt) (U/109 plt) [0.1-0.5] . | RC (U/mL) [> 0.5] . | vWF:CBA (U/mL) [0.8-2.0 × vWF:Ag] . |
|---|---|---|---|---|---|---|
| 0101 (f) | — | — | 1.24 | — | — | 1.09 |
| 0102 (m) | — | — | 0.62 | — | — | 0.59 |
| 0103 | > 12 | 0.06 | 0.07 | 0.06 | — | < 0.01 |
| 0104 (s) | — | — | 0.9 | — | — | 0.93 |
| 0105 (b) | — | — | 0.86 | — | — | 0.9 |
| 0201 (f) | 5 | 0.54 | 0.33 | — | 0.38 | — |
| 0202 (m) | 3 | 0.55 | 0.38 | — | 0.43 | — |
| 0203 | > 20 | 0.12 | < 0.05 | < 0.05 | < 0.05 | — |
| 0303 | > 20 | 0.91 | 1.19 | — | 0.73 | 0.07 |
| Proband . | Bleeding time (min) [< 6] . | FVIII:C (U/mL) [> 0.6] . | vWF:Ag (U/mL) [> 0.5] . | vWF:Ag (plt) (U/109 plt) [0.1-0.5] . | RC (U/mL) [> 0.5] . | vWF:CBA (U/mL) [0.8-2.0 × vWF:Ag] . |
|---|---|---|---|---|---|---|
| 0101 (f) | — | — | 1.24 | — | — | 1.09 |
| 0102 (m) | — | — | 0.62 | — | — | 0.59 |
| 0103 | > 12 | 0.06 | 0.07 | 0.06 | — | < 0.01 |
| 0104 (s) | — | — | 0.9 | — | — | 0.93 |
| 0105 (b) | — | — | 0.86 | — | — | 0.9 |
| 0201 (f) | 5 | 0.54 | 0.33 | — | 0.38 | — |
| 0202 (m) | 3 | 0.55 | 0.38 | — | 0.43 | — |
| 0203 | > 20 | 0.12 | < 0.05 | < 0.05 | < 0.05 | — |
| 0303 | > 20 | 0.91 | 1.19 | — | 0.73 | 0.07 |
The patient with von Willebrand disease (vWD) type 2A, subtype IIE (0103), is from family 01, the patient with vWD type 3 (0203) is from family 02, and 0303 is the patient with vWD 2A, subtype IID. Normal results for each variable are in square brackets. Values for von Willebrand factor: collagen-binding assay (vWF:CBA) are relative to the von Willebrand factor: antigen (vWF:Ag) measured in the same sample. FVIII:C indicates factor VIII coagulant activity; plt, platelet; RC, ristocetin cofactor; f, father of patient; m, mother of patient; s, sister of patient; and b, brother of patient.