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Overwhelmed by New Information on Pathogenesis (and Potential Treatments) of the Antiphospholipid Syndrome
Henry I. Bussey, Pharm.D.
April, 2013
The March 14, 2013 issue of the New England Journal of Medicine has a state of the art article with the deceptively simple title “The Pathogenesis of the Antiphospholipid Syndrome”.1
I suspect that many of us first encountered “the” antiphospholipid antibody syndrome (APAs) in the form of a patient with Lupus Anticoagulant (LAC). Most people who have LAC do not have “Lupus” (systemic lupus erythematosus or SLE), and they have a coagulation – not an anticoagulation – condition. This confusing terminology should have been an early clue to the complexity of this condition – or conditions. In addition, it was something of a mystery as to why some APAs patients seemed much more prone to clots than did others; some had problems with venous clotting while others had arterial clots; and still others had obstetrical problems. This article’s title held out the promise of defining “THE” pathogenesis of THE APAs but, in reality, the APAs is not one condition with a single pathogenesis. APAs – as clearly described in this article – is a complex mix of conditions and our understanding of the pathogenesis is getting more and more complex. This very thorough discussion is packed full of information on an evolving understanding of the many factors that contribute to the APAs; and it discusses multiple new and interesting potential avenues for treatment.
Some of the pathogenic factors discussed (beyond beta2 glycoprotein I antibodies) include the role of endothelial nitric oxide synthase, the A1 – A1 dimer of apolipoprotein E receptor 2, protein disulfide isomerase, the roles of factor XIa and XIIa, tissue factor, complement 5, complement 3, toll-like receptors 7 and 8 (TLR7, TLR8), reactive oxygen species, B cell activating factor (abbreviated BAFF), annexin A2 and annexin A5 (which appear to have opposing effects on clotting), and others. Potentially therapeutic agents discussed include N-acetylcysteine, hydroxychloroquine, the “statins”, inhibitors of factor XIa, belimumab (recently approved to treat SLE but also may reduce thrombosis), and eculizumab. Almost all of this information, and more, is combined into a single color diagram that may be an excellent teaching tool.
On a personal note, the complexity and depth of this paper clearly caused me to recognize the increasing value that I place on being able to call on the expertise of colleagues in hematology and immunology.
Reference
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Giannakopoulos B and Krilis SA. The Pathogenesis of the Antiphospholipid Syndrome. New England Journal of Medicine 2013; 368:1033-44. DOI: 10.1056/NEJMra1112830
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