Factor XIII deficiency
Last updated 18 Aug 2020
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Factor XIII deficiency is a rare bleeding disorder. Researchers have identified an inherited form and a less severe form that is acquired during a person's lifetime.
Description
Factor XIII deficiency is a rare bleeding disorder. Researchers have identified an inherited form and a less severe form that is acquired during a person's lifetime.
Signs and symptoms of inherited factor XIII deficiency begin soon after birth, usually with abnormal bleeding from the umbilical cord stump. If the condition is not treated, affected individuals may have episodes of excessive and prolonged bleeding that can be life-threatening. Abnormal bleeding can occur after surgery or minor trauma. The condition can also cause spontaneous bleeding into the joints or muscles, leading to pain and disability. Women with inherited factor XIII deficiency tend to have heavy or prolonged menstrual bleeding (menorrhagia) and may experience recurrent pregnancy losses (miscarriages). Other signs and symptoms of inherited factor XIII deficiency include nosebleeds, bleeding of the gums, easy bruising, problems with wound healing, bleeding after surgery, and abnormal scar formation. Inherited factor XIII deficiency also increases the risk of spontaneous bleeding inside the skull (intracranial hemorrhage), which is the leading cause of death in people with this condition.
Acquired factor XIII deficiency becomes apparent later in life. People with the acquired form are less likely to have severe or life-threatening episodes of abnormal bleeding than those with the inherited form.
Inheritance patterns
The source lists the following possible inheritance patterns: Autosomal recessive. More than one pattern can apply in different cases.
Related genes
Other names
Deficiency of factor XIII
Deficiency, Laki-Lorand factor
Fibrin stabilizing factor deficiency
Related patient information and medicines
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Blood clotting tests
Blood clotting tests are used to diagnose and assess bleeding problems and to monitor people who take warfarin or other anticoagulant medicines. You may be advi... These chemicals then react with other chemicals and proteins in the plasma, called clotting factors. There are 13 known clotting factors which are called by their Roman numbers - factor.
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Further reading and references
- Factor XIII deficiency; MedlinePlus, National Library of Medicine.
- Biswas A, Ivaskevicius V, Thomas A, Oldenburg J. Coagulation factor XIII deficiency. Diagnosis, prevalence and management of inherited and acquired forms. Hamostaseologie. 2014;34(2):160-6. doi: 10.5482/HAMO-13-08-0046. Epub 2014 Feb 7.
- de Jager T, Pericleous L, Kokot-Kierepa M, Naderi M, Karimi M. The burden and management of FXIII deficiency. Haemophilia. 2014 Nov;20(6):733-40. doi: 10.1111/hae.12474. Epub 2014 Jul 17.
- Kohler HP, Ichinose A, Seitz R, Ariens RA, Muszbek L; Factor XIII And Fibrinogen SSC Subcommittee Of The ISTH. Diagnosis and classification of factor XIII deficiencies. J Thromb Haemost. 2011 Jul;9(7):1404-6. doi: 10.1111/j.1538-7836.2011.04315.x. No abstract available.
- Levy JH, Greenberg C. Biology of Factor XIII and clinical manifestations of Factor XIII deficiency. Transfusion. 2013 May;53(5):1120-31. doi: 10.1111/j.1537-2995.2012.03865.x. Epub 2012 Aug 28.
- Muszbek L, Bagoly Z, Cairo A, Peyvandi F. Novel aspects of factor XIII deficiency. Curr Opin Hematol. 2011 Sep;18(5):366-72. doi: 10.1097/MOH.0b013e3283497e3e.
- Schroeder V, Kohler HP. Factor XIII deficiency: an update. Semin Thromb Hemost. 2013 Sep;39(6):632-41. doi: 10.1055/s-0033-1353392. Epub 2013 Aug 8.
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