Concomitant hemostatic abnormalities identified
| Concomitant hemostatic defect . | n . | Sex . | ISTH BAT score . | Factor level (IU/dL) . | Normal reference range (IU/dL) . |
|---|---|---|---|---|---|
| Factor deficiency | |||||
| Factor V | 1 | M* | 11 | 30 | 70-137 |
| Factor VII | 2 | F, F | 10, 17 | 31, 39 | 70-170 |
| Factor VIII (confirmed carrier) | 2 | F, F* | 17, 14 | 28, 39 | 60-136 |
| Factor XI | 2 | M, F | 5, 8 | 50, 58 | 72-152 |
| Factor XIII | 1 | F | 17 | 48 | 73-160 |
| Platelet functional defect | |||||
| No response to adrenaline | 1 | M* | 11 | ||
| Reduced response to ADP and adrenaline | 1 | F | 7 | ||
| Reduced response to AA and adrenaline | 1 | F | 8 | ||
| One-phase ADP | 1 | F* | 14 |
| Concomitant hemostatic defect . | n . | Sex . | ISTH BAT score . | Factor level (IU/dL) . | Normal reference range (IU/dL) . |
|---|---|---|---|---|---|
| Factor deficiency | |||||
| Factor V | 1 | M* | 11 | 30 | 70-137 |
| Factor VII | 2 | F, F | 10, 17 | 31, 39 | 70-170 |
| Factor VIII (confirmed carrier) | 2 | F, F* | 17, 14 | 28, 39 | 60-136 |
| Factor XI | 2 | M, F | 5, 8 | 50, 58 | 72-152 |
| Factor XIII | 1 | F | 17 | 48 | 73-160 |
| Platelet functional defect | |||||
| No response to adrenaline | 1 | M* | 11 | ||
| Reduced response to ADP and adrenaline | 1 | F | 7 | ||
| Reduced response to AA and adrenaline | 1 | F | 8 | ||
| One-phase ADP | 1 | F* | 14 |
Patients with low VWF levels and significant bleeding histories (n = 91) were screened for additional coagulation disorders (see “Methods” for details). Concomitant hemostatic abnormalities were identified in only 10 individuals (in 2 subjects, combined platelet function defects and coagulopathies were found).
Combined mild factor deficiency and platelet function defect.
AA, arachdonic acid; ADP, adenosine 5′-diphosphate.