Factor X deficiency is an inherited bleeding disorder that is caused by a problem with factor X. Because the body produces less factor X than it should, or because the factor X is not working properly, the clotting reaction is blocked prematurely and the blood clot does not form.
Factor X deficiency is an autosomal recessive disorder, which means that both parents must carry the defective gene in order to pass it on to their child. It also means that the disorder affects both males and females. Factor X deficiency is one of the rarest inherited clotting disorders, but like all autosomal recessive disorders, it is found more frequently in areas of the world where marriage between close relatives is common.
Factor X deficiency may also be inherited with other factor deficiencies (see “Combined deficiency of vitamin K-dependent clotting factors”).
As a general rule, the less factor X a person has in his/her blood, the more frequent and/or severe the symptoms. People with severe factor X deficiency can have serious bleeding episodes.
- Nosebleeds (epistaxis)
- Easy bruising
- Bleeding in the gut (gastrointestinal haemorrhage)
- Bleeding into joints (hemarthrosis)
- Muscle bleeds
- Bleeding from the umbilical cord stump at birth
- Bleeding from the mouth, particularly after dental surgery or tooth extraction
- Bleeding during or after surgery or injury
Other reported symptoms
- Heavy or prolonged menstrual bleeding (menorrhagia)
- Bleeding after circumcision
- Abnormal or prolonged bleeding after childbirth
- First-trimester miscarriage (spontaneous abortion)
- Blood in the urine (haematuria)
- Bleeding in the central nervous system (the brain and spinal cord)
Factor X deficiency is diagnosed by a variety of blood tests that should be performed by a specialist at your Comprehensive Care Centre.
There are two treatments available for factor X deficiency. Both are made from human plasma.
- Prothrombin Complex Concentrate, (PCC) containing factor X
- Fresh Frozen Plasma (FFP)
Excessive menstrual bleeding in women with factor X deficiency may be controlled with hormonal contraceptives (birth control pills) or antifibrinolytic drugs.