Thrombotic thrombocytopenic purpura and the atypical hemolytic uremic syndrome: an update

Hematol Oncol Clin North Am. 2013 Jun;27(3):565-84. doi: 10.1016/j.hoc.2013.02.006.


Since the last review in 2007 of thrombotic thrombocytopenic purpura (TTP) and microangiopathic hemolytic anemia in the Clinics, further understanding of the nature of TTP and atypical hemolytic uremic syndrome (aHUS) has led to increasing use of rituximab in the treatment of TTP and the approval in 2011 of eculizumab for the treatment of aHUS. With this new armamentarium, distinction of aHUS from TTP has become more critical than ever. This article updates the new knowledge, highlights the difference between aHUS and TTP, and presents a scheme for their diagnosis and management.

Publication types

  • Case Reports
  • Review

MeSH terms

  • ADAM Proteins
  • ADAMTS13 Protein
  • Adult
  • Animals
  • Atypical Hemolytic Uremic Syndrome
  • Diagnosis, Differential
  • Female
  • Hemolytic-Uremic Syndrome / diagnosis*
  • Hemolytic-Uremic Syndrome / etiology*
  • Hemolytic-Uremic Syndrome / therapy
  • Humans
  • Male
  • Middle Aged
  • Purpura, Thrombotic Thrombocytopenic / diagnosis*
  • Purpura, Thrombotic Thrombocytopenic / etiology*
  • Purpura, Thrombotic Thrombocytopenic / therapy


  • ADAM Proteins
  • ADAMTS13 Protein
  • ADAMTS13 protein, human