What is the evidence for the role of therapeutic apheresis in the management of complement-associated thrombotic microangiopathies?

Yildiz S., DEMİRKAN F.

TRANSFUSION AND APHERESIS SCIENCE, vol.57, no.1, pp.31-34, 2018 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Review
  • Volume: 57 Issue: 1
  • Publication Date: 2018
  • Doi Number: 10.1016/j.transci.2018.02.013
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.31-34
  • Keywords: Thrombotic microangiopathies, Alternative complement system, Complement-mediated thrombotic, microangiopathies, Therapeutic apheresis, HEMOLYTIC-UREMIC SYNDROME, TOXIN-ASSOCIATED HUS, THROMBOCYTOPENIC PURPURA, ALTERNATIVE PATHWAY, PATHOPHYSIOLOGY, AUTOANTIBODIES, ECULIZUMAB, ACTIVATION, MUTATIONS, DISEASES
  • Dokuz Eylül University Affiliated: Yes


Thrombotic microangiopathies (TMAs) are disorders characterized by endothelial cell activation, microangiopathic hemolytic anemia, thrombocytopenia and organ failure of variable intensity. The pathophysiology of various types of TMAs have become an interesting field of study. Alternative complement system activation plays an important role in several pathophysiological conditions. Complement activation is also described in an increasing number of TMAs. Inherited defects in complement regulatory genes and acquired autoantibodies against complement regulatory proteins have been described. Atypical hemolytic uremic synrome (HUS) is caused by uncontrolled activation of the alternative complement system, now called complement-mediated TMAs. Recently, application of a monoclonal antibody that specifically binds to C5 became available to treat patients with complement-mediated TMAs. Eculizumab is a humanized monoclonal antibody that blocks complement C5 activation. Empiric therapeutic apheresis is also recommended in all forms of complement-mediated TMAs. The justification for therapeutic apheresis use in all forms of complement-mediated TMAs is that it can effectively remove the autoantibodies or mutated circulating complement regulators while replacing absent or defective complement regulators. Currently, therapeutic apheresis and eculizumab are the available treatment options for complement-mediated TMAs. In this paper, we review the evidence for the role of therapeutic apheresis in the management of complement-associated TMAs. (C) 2018 Elsevier Ltd. All rights reserved.