von Willebrand disease | | | | | | |
Type 1 | vWF level decreased | Mild to moderate | Increase vWF-containing concentrates or desmopressin | vWF-containing factor VIII concentrate | ≤ 4 h/1–3 d |
Once per day Yes if prior > 12 h
|
Type 2 | Dysfunctional vWF | Variable/moderate | Small increase in vWF-containing concentrates | vWF-containing factor VIII concentrate | ≤ 4 h/1–3 d |
Once per day Yes if prior > 12 h
|
Type 3 | vWF absent | Severe | No increase in vWF-containing concentrates | vWF-containing factor VIII concentrate | ≤ 2 h/> 3–5 d |
Once or twice per day Yes if prior > 8 h
|
Hemophilia A | Factor VIII level decreased | Mild to moderate, with factor VIII level < 40 IU/dL |
| Recombinant factor VIII | ≤ 2 h/> 3–5 d |
Once or twice per day Yes if prior > 8 h
|
Hemophilia B | Factor IX level decreased | Mild to moderate, with factor IX level < 40 IU/dL |
| Recombinant factor IX | ≤ 2 h/> 3–5 d |
Once per day Yes if prior > 8 h
|
Glanzmann thrombasthenia | Platelet function disorder | Associated with severe bleeding phenotype |
| — | — | |
Bernard–Soulier syndrome | Platelet receptor abnormality | Associated with severe bleeding phenotype |
| — | — | |
Factor XI deficiency | Factor XI level decreased | Highly variable but increased with factor XI level < 15 IU/dL |
|
| ≤ 8 h/1–3 d |
Once per day Yes if prior > 12 h
|