Hemophilia Association of New Jersey

Single Factor Deficiency

There are ten clotting factors that are necessary in forming a blood clot. Deficiencies in factors VIII and IX are well known to most people, but what of the other factor deficiencies? Not everyone is as familiar with these conditions because they are diagnosed so rarely To date, deficiencies in eight of the lesser known coagulation factors (Factors I, II, V, VII, X, XI, XII and XIII) have been documented in the medical literature. Many of these disorders were only discovered or described within the last 40 years.

In most cases, rare factor deficiencies are not genetically sex-linked. They occur in equal frequency among men and women. By and large the gene is passed down in an autosomal recessive fashion. This means that when the factor deficiency is inherited from only one parent, the child will be a carrier of the condition, but usually not have symptoms. It is possible for people to inherit a gene from both parents, but this happens very rarely and usually means a more severe manifestation of the disease.

Not all factor deficiencies have the same severity. Not everyone with these disorders needs treatment. However for those who do, the treatments available for people with rare factor deficiencies are not optimal. Many people in the United States with rare factor deficiencies need to take fresh frozen plasma, prothrombin complex concentrates (PCCs) or cryoprecipitate.

Some of the rarer factor deficiencies (such as Factor VII and XIII) do have other treatment options. As always, consult your hemophilia treatment center physician.

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