<unk> ,<unk> , also Asherson’s<unk> , is an acute and complex biological process that leads to occlusion of small vessels of various organs. It was first described by Ronald Asherson in 1992. The<unk>  exhibits thrombotic microangiopathy, multiple organ failure, and tissue necrosis and is considered an extreme variant of the antiphospholipid<unk> .<unk>  has a mortality rate of about 50 %. With the establishment of a<unk> -Registry more has been learned about this<unk> , yet the cause remains elusive. Infection, trauma, medication, or surgery can be identified in about half the cases as a “trigger”. It is thought that cytokines are activated leading to a cytokine storm with the potentially fatal consequences of organ failure. Patients exhibit a positive test to antilipid antibodies, typically IgG, and often have a history of lupus or another connective tissue disease. Thrombocytopenia is a common finding.
Clinically, the<unk>  can affect many organs systems. Peripheral thrombosis may be encountered affecting veins or arteries. Intraabdominal thrombosis may lead to pain. Renal and pulmonary complications are common. The patient may exhibit skin purpura and necrosis. Cerebral manifestations may lead to encephalopathy and seizures.  Myocardial infarctions may occur. Death may result from multiple organ failure.
Treatments may involve the following steps:
Prevention includes the use of antibiotics for infection and parenteral anticoagulation for susceptible patients.
Specific therapy includes the use of  intravenous heparin, corticosteroids, and possibly plasma exchanges, intravenous immunoglobulin.
Additional steps may have to be taken to manage circulatory problems, renal failure, and respiratory distress.
Catastrophic antiphospholipid syndrome
Antiphospholipid syndrome
Factor V Leiden
Protein C deficiency
Antithrombin III deficiency
Giant platelet disorder
Activated protein C resistance
Protein S deficiency