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

N. Besbas, D. Karpman, D. Landau, C. Loirat, W. Proesmans, G. Remuzzi, G. Rizzoni, C. M. Taylor, N. Van De Kar, L. B. Zimmerhackl

Research output: Contribution to journalReview articlepeer-review

236 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)423-431
Number of pages9
JournalKidney International
Volume70
Issue number3
DOIs
StatePublished - 21 Aug 2006
Externally publishedYes

Keywords

  • Etiology
  • Hemolytic uremic syndrome
  • Thrombotic thrombocytopenic purpura

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