Haemophilia B
Haemophilia B is due to a deficiency of Factor IX. It is also known as Christmas Disease (after the person in whom the disease was first identified). The genetic defect is sex-linked and approximately 1 in 50,000 are affected. If untreated, patients with severe deficiencies may have frequent spontaneous bleeding into joints and muscles; they bleed excessively during minor surgery (e.g. tooth extraction) and may suffer life-threatening bleeding from trauma or even relatively mild injury. Replacement of the missing Factor IX corrects the bleeding problem.
Correlation of Coagulation Factor activity
and disease severity
in Haemophilia A and B
| Coagulation Factor Activity (% normal) | Clinical Manifestation |
|---|---|
| <2 | Severe – frequent spontaneous bleeds from early life, joint deformity and crippling if not adequately treated |
| 2-10 | Moderate – post-traumatic bleeding, occasional spontaneous bleeds |
| >10 | Mild – post-traumatic bleeding |
Patient Information Leaflets (PIL)
Factor IX Concentrate - Patient Information Leaflet (PIL)
Support Information
Haemophilia - Issues and options for women who are carriers.
A comprehensive guide designed to answer your questions and offer reassurance that, in many cases, carriers of haemophilia can be helped and supported and the condition treated.
Mild or Moderate Haemophilia - How will it affect my life?
An introduction to the disorder for those newly diagnosed, giving information
on what it is, how it will affect them and how it can be managed.
von Willebrand Disease - How will it affect my life?
An introduction to the disorder for those newly diagnosed, giving information on what it is, how it will affect them and how it can be managed.
For further information, please contact your GP.
