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Open Access Highly Accessed Research article

Thrombocytopenia is not mandatory to diagnose haemolytic and uremic syndrome

Marion Sallée1, Khalil Ismail1, Fadi Fakhouri2, Henri Vacher-Coponat1, Julie Moussi-Francés1, Véronique Frémaux-Bacchi3 and Stéphane Burtey1*

Author Affiliations

1 APHM, Hopital de la Conception, centre de néphrologie et transplantation rénale, Aix-Marseille Univ, Marseille, 13005, France

2 Département de Nephrologie, INSERM UMR643, CHU de Nantes, 1 place Alexis-Ricordeau, Nantes, 44000, France

3 Service d’immunologie biologique, hôpital Européen Georges-Pompidou, Assistance publique–Hôpitaux de Paris, 20-40, rue Leblanc, Paris cedex 15, 75908, France

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BMC Nephrology 2013, 14:3  doi:10.1186/1471-2369-14-3

Published: 8 January 2013

Abstract

Background

Hemolytic and uremic syndrome (HUS) diagnosis involves association of non immune hemolytic anemia, thrombocytopenia, and renal failure. HUS without thrombocytopenia has been observed, we call it partial HUS. Its real frequency and outcome are unknown. The aim of this study was to determine the prevalence of patients with normal platelets count in two HUS cohorts and to compare their outcome to patients with thrombocytopenia.

Methods

We retrospectively identified HUS diagnosis in two different cohorts. The first cohort was from a single center and consisted of all cases of HUS whatever the aetiology, the second was multicentric and consisted of atypical HUS patients. These cohorts were divided into two groups depending on the presence or absence of thrombocytopenia. Clinical and biological data were compared between thrombopenic and non thrombopenic group.

Results

We identified 13% (20/150) of patients with normal platelets count: 10 episodes (18%) of HUS in six patients (14%) in the monocentric cohort and 14 patients (13%) with 17 episodes (12%) in the multicentric cohort of atypical HUS. Groups differed in platelets count and LDH level. In both cohorts, renal outcome was similar to patient presenting with thrombocytopenia.

Conclusion

HUS with normal platelets count is not infrequent. Relative to classical clinical presentation of HUS, partial HUS has similar characteristics and identical poor renal outcome and so must be treated in the same way.

Keywords:
Hemolytic and uremic syndrome; Thrombocytopenia; Complement