Eculizumab proves effective in treating atypical hemolytic uremic syndrome

ECULIZUMAB


A new treatment for patients with atypical hemolytic uremic syndrome ( aHUS) was tested by researchers at Emory University. According to an article published in the New England Journal of Medicine, it seems that eculizumab, a monoclonal antibody, is effective in the management of this life-threatening inflammatory disease.
 
Hemolytic uremic syndrome, which is a thrombotic microangiopathy that causes blood clots in small vessels, is characterized by thrombocytopenia, hemolytic anemia and uremia. It mostly affects children up to 7 years old and is the most common cause of acute renal failure in children. The hemolytic uremic syndrome is often associated with enteric infections (E. coli, Shigella, Salmonella, etc.), but it can occur in other situations such as after certain drugs, tumors, after transplantation, etc..

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Site of action of eculizumab. a Complement C5 is split by C5 convertase into C5a and C5b. C5a increases the permeability of blood vessels and attracts inflammatory cells by chemotaxis. C5b binds to other complement components (C6, C7, and C8). The C5b-8 complex is expanded with C9 to form the MAC. MAC binds and permeabilizes bacterial walls (e.g. Neisseria), thereby killing the microorganism. b Eculizumab is a long-acting humanized monoclonal antibody targeted against complement C5. It inhibits the cleavage of C5 into C5a and C5b and hence inhibits deployment of the terminal complement system including the formation of MAC