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Factor XIII Deficiency
 

(Can also be known as Fibrin Stabilizing Factor deficiency)

This condition is perhaps the rarest of all factor deficiencies.  The incidence of Factor XIII deficiency is estimated at one in five million births.  It is inherited in an autosomal recessive fashion, which means it affects men and women equally.  No racial or ethnic group is disproportionately affected. 

Factor XIII is the protein responsible for stabilizing the formation of a blood clot.  In the absence of Factor XIII, a clot will still develop but it will remain unstable. When someone has a deficiency of Factor XIII, the tenuously formed clot will eventually break down and cause recurrent bleeds.  The prolonged bleeding that is associated with Factor XIII is usually associated with trauma. Among severe patients there is a high risk of head bleeds with or without trauma. Bleeding immediately after surgery is usually not excessive, but can be delayed.  Women who go untreated risk spontaneous abortion.  Men with the deficiency may show signs of infertility.  Common characteristics include soft tissue bleeds, menorrhagia, joint bleeding, and persistent bleeding during circumsicion or at the site of the umbilical cord. 

Diagnosis is made by normal coagulation screening tests and a detailed family history.  Specific factor XIII assays can confirm the diagnosis.  The condition can also be defined by a clot solubility test. 

Treatment

In February 2011, the US Food and Drug Administration approved Corifact, a product manufactured by CSL Behring of Marburg, Germany, to prevent bleeding in people with congenital Factor XIII deficiency. 

Corifact is made from the pooled plasma of healthy donors. It can be used for patients with absent or decreased levels of FXIII. 

People receiving Corifact may develop antibodies against Factor XIII that may make the product ineffective. It potentially can cause adverse events from abnormal clotting if doses higher than the labeled dose are given to patients.  Cryoprecipitate should not be used to treat patients with factor XIII deficiency except in life- and limb-threatening emergencies when Factor XIII concentrate is not immediately available.

 

 

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