coagulation factors

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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 minor injury. Replacement of the missing Factor IX corrects the bleeding problem.

Table 1 - Correlation of coagulation factor activity and severity in Haemophilia A and B

Coagulation Factor Activity (% normal*)

Clinical Manifestation

≤1

Severe - frequent spontaneous bleeds from early life, joint deformity and crippling if not adequately treated

>1 to <5

Moderate - post-traumatic bleeding, occasional spontaneous bleeds

≥5

Mild - post-traumatic bleeding

 

* Normal is 100% and is defined as 100 international units (IU) of the factor per dL of blood. 'Normal' coagulation factor activity can range from 50 - 200%. About one-third of carriers have low or subnormal activity. In normal pregnancy, activity levels may exceed 200%.


Useful documentation available from the electronic Medicines Compendium (eMC).

PDF High Purity Factor IX Concentrate (Patient Information Leaflet)

Patient information leaflets