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Does hemolytic uremic syndrome differ from thrombotic thrombocytopenic purpura?

Nature Clinical Practice Nephrology volume 3, pages 679–687 (2007)Cite this article

Abstract

Both hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are characterized by thrombotic microangiopathy (TMA), affecting mainly the kidney and brain, respectively. Diagnosis of HUS or TTP has been complicated by the fact that these disorders share several clinical characteristics, and by the dearth of knowledge regarding the pathogenesis of TMA. Advances in the identification of pathogenic features—deficiency of the metalloprotease ADAMTS13 in TTP and association of mutated complement proteins with atypical HUS—have gone some way towards improving clinicians' ability to distinguish between the two diseases. Here, we pose the following question: is it important to patient management that HUS be distinguished from TTP? By discussing what is known about the pathogenesis, clinical features and treatment of these two conditions we address this question, and propose a new nomenclature for TMA.

Key Points

  • The primary sites of thrombotic microangiopathy (TMA) in hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are the kidney and brain, respectively

  • Distinguishing between HUS and TTP on the basis of simple, dichotomous clinical criteria (HUS, mild thrombocytopenia and a serum creatinine level >200 µmol/l [2.3 mg/dl]; TTP, severe thrombocytopenia and serum creatinine level <120 µmol/l [1.4 mg/dl]) is problematic as there is often overlap in clinical presentation between patients with the two disorders

  • Recent pathogenic insights (association of mutated complement proteins with atypical HUS and deficiency of the metalloprotease ADAMTS13 in TTP) have made it easier to differentiate between HUS and TTP

  • Adopting a new nomenclature—'complement-dysregulation-related TMA' and 'ADAMTS13-deficiency-related TMA', in place of 'TTP' and 'HUS', respectively—might facilitate more-effective clinical management of patients

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Figure 1: Light microscopic images of kidney biopsy tissue from a patient with 'complement-dysregulation-related TMA' (hemolytic uremic syndrome; HUS) and severe renal failure.
Figure 2: Scheme of physiological processes leading to TMA.

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Author notes

  1. V Frémeaux-Bacchi is a Clinical Researcher in the Department of Immunology, Hôpital Européen Georges Pompidou, Paris, France.

Authors and Affiliations

  1. F Fakhouri is a Clinical Researcher in the Department of Nephrology, Université Paris Descartes, Assistance Publique - Hôpitaux de Paris, Hôpital Necker, Paris, France.,

    Fadi Fakhouri & Véronique Frémeaux-Bacchi

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Correspondence to Fadi Fakhouri.

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Fakhouri, F., Frémeaux-Bacchi, V. Does hemolytic uremic syndrome differ from thrombotic thrombocytopenic purpura?. Nat Rev Nephrol 3, 679–687 (2007). https://doi.org/10.1038/ncpneph0670

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