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Asherson's Syndrome

Abstract

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NORD is very grateful to Ronald A. Asherson, MD, deceased, former Professor of Immunology, University of the Witwatersrand, and Consultant Rheumatologist, The Rosebank Clinic, both in Johannesburg, South Africa, for assistance in the preparation of this report.

Synonyms of Asherson's Syndrome

  • Catastrophic Antiphospholipid Syndrome

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Asherson's syndrome is an extremely rare autoimmune disorder characterized by the development, over a period of hours, days or weeks, of rapidly progressive blood clots affecting multiple organ systems of the body. Conditions such as infections, immunizations, wounds caused due to physical trauma and failure in the anticoagulation mechanism of the body usually act as "triggers".

The syndrome is particularly common among patients with antiphospholipid syndrome who experience a cessation of the anticoagulation mechanism rleated to recurrent bleeding in the body. It is usually seen in patients who have previously suffered from a simple/classic episode of antiphospholipid syndrome. It is not known why patients of antiphospholipid syndrome often have the tendency to be "catapulted" into a serious or fatal multiorgan failure, while the same triggers in other individuals may only result in recurrent large vessel thrombosis. The symptoms are also aptly observed in patients during pregnancy or in the weeks after childbirth (puerperium) and may follow the HELLP syndrome or be associated with malignancies. Symptoms vary from case to case depending upon the specific organ systems involved. Asherson's syndrome can rapidly result in life-threatening multiorgan failure.

Asherson's syndrome is a severe variant of antiphospholipid syndrome (APS), an autoimmune disorder in which blood clots occur in relation to the presence of antiphospholipid antibodies in the body. Antibodies are specialized proteins produced by the body's immune system to fight infection. In autoimmune disorders, antibodies mistakenly attack healthy tissue. In APS and Asherson's syndrome, antibodies mistakenly attack certain proteins that bind to phospholipids, which are fat molecules that are involved in the proper function of cell membranes. Phospholipids are found throughout the body. The reason these antibodies attack these proteins and the process by which they cause blood clots to form is not known.

Asherson's syndrome may occur in individuals who have primary or secondary APS or in individuals with lupus or other autoimmune disorders. In some cases, no previous history of these disorders may be present. The exact cause of Asherson's syndrome is unknown.

Organizations related to Asherson's Syndrome

Please note that some of these organizations may provide information concerning certain conditions potentially associated with this disorder.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

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