A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders

Research output: Contribution to journalReview article


The diagnostic terms hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are based on historical and overlapping clinical descriptions. Advances in understanding some of the causes of the syndrome now permit many patients to be classified according to etiology. The increased precision of a diagnosis based on causation is important for considering logical approaches to treatment and prognosis. It is also essential for research. We propose a classification that accommodates both a current understanding of causation (level 1) and clinical association in cases for whom cause of disease is unclear (level 2). We tested the classification in a pediatric disease registry of HUS. The revised classification is a stimulus to comprehensive investigation of all cases of HUS and TTP and is expected to increase the proportion of cases in whom a level 1 etiological diagnosis is confirmed.


  • N. Besbas
  • Diana Karpman
  • D. Landau
  • C. Loirat
  • W. Proesmans
  • G. Remuzzi
  • G. Rizzoni
  • C. M. Taylor
  • N. Van de Kar
  • L. B. Zimmerhackl
Research areas and keywords

Subject classification (UKÄ) – MANDATORY

  • Urology and Nephrology


  • hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, etiology
Original languageEnglish
Pages (from-to)423-431
JournalKidney International
Issue number3
Publication statusPublished - 2006
Publication categoryResearch