Protein S deficiency
| Classification according to ICD-10 | |
|---|---|
| D68.5 | Primary thrombophilia |
| ICD-10 online (WHO version 2019) | |
Protein S deficiency is a congenital or acquired blood disorder with a significantly increased risk of leg vein thrombosis .
description
Free Protein S is located in the human blood plasma and is an endogenous, vitamin K-dependent anticoagulant that as a non-enzymatic cofactor in the proteolysis of factor Va and coagulation Factor VIII , the protein C activated. A protein S deficiency leads to a reduced inactivation of the coagulation factors Va and VIIIa and thus an increased tendency to venous thrombosis.
Protein S deficiency can lead to disseminated intravascular coagulopathy , deep vein thrombosis, or pulmonary embolism and can be fatal.
causes
If the cause of the protein S deficiency is a genetic defect, there is a fifty percent probability of first-degree relatives being inherited, regardless of gender .
Protein S deficiency can also be caused by vitamin K deficiency, the use of vitamin K antagonists or ovulation inhibitors , chronic infections, or liver disease . Further, a come inflammation , a sepsis , a burn , a trauma or a large operation as the cause in question.
Very rarely, a deficiency can occur in the context of a syndrome : Hereditary combined deficiency of vitamin K-dependent coagulation factors .
treatment
After a thrombosis has occurred, therapy with vitamin K antagonists, such as phenprocoumon, is carried out .
New oral anticoagulants (NOAC) are not approved for treatment.
classification
A distinction is made between three types of congenital protein S deficiency:
- Type I: Decreased protein S activity with decreased total protein S and decreased free protein S
- Type II: Decreased protein S activity with normal total protein S and normal free protein S
- Type III: Decreased protein S activity with normal total protein S and decreased free protein S
Individual evidence
- ↑ Deficiency of the body's own anticoagulants antithrombin, protein C, protein S - how about heredity? , die-herzklappe.de, accessed online on March 10, 2013
- ↑ Christoph Marschall: Hereditary Protein S Deficiency ( Memento from September 3, 2012 in the Internet Archive ), Center for Human Genetics and Laboratory Medicine, Martinsried, accessed on April 28, 2016
Web links
- Christoph Marschall: Hereditary Protein S Deficiency ( Memento from September 3, 2012 in the Internet Archive ), Center for Human Genetics and Laboratory Medicine, Martinsried
- Deficiency of the body's own anticoagulants antithrombin, protein C, protein S , working group for coagulation and heart valve patients, Ratingen
