Antiphospholipid Syndrome
Hughes Syndrome (APS)
In the antiphospholipid syndrome are immunoglobulins (APLA) states which are directed against a complex in the coagulation cascade. Thus the process of coagulation in the body (will be inhibited to varying degrees).
These so-called Lupus anticoagulants not lead to an increased bleeding tendency, but increase the probability of developing thrombosis disease to many times. In women, increases the rate of miscarriage and premature births.
If there is such an event and can be detected in the blood, the antibodies described above, we speak of an antiphospholipid syndrome.
Frequency
About three percent of the population are APLA positive, of which approximately 40% are symptomatic.
Symptoms and Complications:
- Thrombosis embolism, which can lead to heart attacks or strokes (Significantly increased Risk comparison the general population).
- Degradation of platelets in the blood (thrombocytopenia)
- Pregnancy complications, early abortion and births
- Necrosis of the skin by "clogging" of small vessels
Diagnosis
- The detection of antibodies
- Screening test (determination of the kaolin-PTT-Clotting Time and the diluted Russell Viper Venom Time)
- Confirmatory tests (plasma exchange and platelet neutralization test)
- If a screening test and two confirmatory tests positive, the diagnosis of antiphospholipid syndrome assured.
Therapy
- Long-term anticoagulation to prevent thrombosis
- at an extremely pronounced APS is a possibility, a therapeutic trial with a try to plasmapheresis
- in patients without symptoms no treatment is necessary, but should of situations which are a special risk of thrombosis (bed rest, etc.) made a prophylaxis