Classification | F VIII activity, % of normal (60-160%) | Clinical picture | Age at diagnosis (in the absence of known family history) |
---|---|---|---|
Severe | < 1 | Spontaneous intra-articular bleeding (80%) Extensive soft tissue bleeding (10-20%) Other severe bleeding (5-10%) Cerebral haemorrhage (< 5%) | Below 1 year |
Moderately severe | 1-5 | Incidental spontaneous soft tissue or intra-articular bleeding Significant bleeding in association with trauma or surgery | 1-7 years |
Mild | > 5 | Severe bleeding in association with major trauma or surgery | Often later in life |
Disease | Type of disease | Treatment of mild bleeding | Treatment of severe bleeding |
---|---|---|---|
Haemophilia A | Mild | Tranexamic acid, DDAVP | DDAVP or F VIII product |
Moderately severe to severe | F VIII product: at least 20-40 units/kg; after 12-24 h, 1-2 additional doses | F VIII product
| |
Haemophilia B | Mild, moderately severe or severe | F IX product: 30-50 units/kg | F IX product: initial dose 80-120 units/kg (target F IX level more than 50-80%) |
vWD | Type 1 (mild) | Mucosal bleeding: 20-25 mg/kg tranexamic acid 3 times daily p.o. | DDAVP or FVIII/vWF or vWF product |
Other bleeding: tranexamic acid + DDAVP | F VIII/vWF or vWF product | ||
Type 2 (moderately severe) | Tranexamic acid + DDAVP or F VIII/vWF product | F VIII/vWF or vWF/F VIII product | |
Type 3 (severe) | vWF/F VIII product | F VIII/vWF or vWF/F VIII product |