Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome in 2014—Current Knowledge and Outcomes with Plasma Exchange

Abstract

Great progress has been made in our understanding of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome since Moschowitz first described this entity in 1925. This review provides a contemporary insight into the pathophysiology, diagnosis, and classification of these disorders in both adults and children. Lessons learned from major worldwide registry data and disease epidemics, including the 2011 German outbreak, are discussed with recommendations for management of specific clinical conditions based on available evidence, including the role of plasma exchange, rituximab, and eculizumab.

Plasmapheresis for the treatment of kidney diseases

“…18 Introduction of the diagnostic dyad (unexplained thrombocytopenia and microangiopathic hemolytic anemia) expanded the application of plasma exchange to prevent early mortality within the thrombotic microangiopathy spectrum. [19][20][21] The superiority of plasma exchange over infusion was thought to be due to removal of a mystery substance and provision of a necessary/deficient substance. In 1998, 2 different laboratories identified the mystery substance as an antibody inhibitor to the ADAMTS13 enzyme, and the deficient substance as the ADAMTS13 enzyme, which cleaves von Willebrand factor multimers and prevents widespread microthrombosis.…”