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Rare Bleeding Disorders

There are a number of related bleeding disorders where there are other factor deficiencies that are much more rare than haemophilia. The effects will depend, like haemophilia, on the degree of the missing factor. Without treatment, prolonged bleeding may follow surgery, childbirth, or major injury.

With haemophilia A there is a factor VIII deficiency, with haemophilia B there is a factor IX deficiency and with von Willebrands disorder there is a von Willebrand factor (and sometimes factor VIII) deficiency. These are some of the other rare bleeding disorders that have been identified.

Factor I (fibrinogen) deficiency

Joint bleeds are uncommon, but there is more likelihood of oozing from small wounds than in haemophilia. Easy bruising will occur.

Factor II (prothrombin) deficiency

Nosebleeds, heavy periods, and easy bruising are features. Joint bleeds are rare.

Factor V deficiency

Severely affected people experience easy bruising, nosebleeds, heavy periods, and joint and muscle bleeds.

Factor VII deficiency

Effects are usually mild but include nosebleeds, heavy periods, and bruising. Joint bleeds are rare.

Factor X deficiency

Bruising, joint bleeds, and heavy periods occur in people with this deficiency.

Factor XI deficiency

Also known as haemophilia C, factor XI helps activate factor IX. Nosebleeds and heavy periods can occur.

Factor XII deficiency

This deficiency is usually diagnosed only when blood taken for some other reason fails to clot in the normal time. Surprisingly, people with even a severe deficiency have little or no trouble from bleeding.

Factor XIII deficiency

Clots form in the normal time but tend to break down later as they are not stabilised. This leads to a delay in healing after injury or surgery. Females with severe XIII deficiency have frequent miscarriages. Factor XIII deficiency may present early in life with oozing from the umbilical cord. People with severe factor XIII deficiency should be treated with monthly prophylaxis with factor XIII concentrate. This prevents bleeding altogether.

Glanzmann Thrombasthenia

Glanzmann Thrombasthenia affects both men and women and can range from mild to severe. Diagnostic features are a normal platelet count with a prolonged bleeding time. Bruising, nosebleeds and mouth bleeds can occur and can be severe. Women and girls can have heavy periods.

Bernard-Soulier Syndrome

In people with Bernad-Soulier Syndrome platelets lack the ability to adhere to the walls of injured blood vessels, which means an adequate blood clot cannot be formed. Symptoms are nose and mouth bleeds, bruising, bleeding after trauma and heavy periods in girls and women.

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