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NDT Advance Access published online on December 18, 2008
Nephrology Dialysis Transplantation, doi:10.1093/ndt/gfn687
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© The Author [2008].
The online version of this article has been published under an open access model. Users are entitled to use, reproduce, disseminate, or display the open access version of this article for non-commercial purposes provided that: the original authorship is properly and fully attributed; the Journal and Oxford University Press are attributed as the original place of publication with the correct citation details given; if an article is subsequently reproduced or disseminated not in its entirety but only in part or as a derivative work this must be clearly indicated. For commercial re-use, please contact journals.permissions@oxfordjournals.org


Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria

Sacha A. De Serres and Paul Isenring

The Nephrology Research Group, L’Hôtel-Dieu de Québec Research Institution, Department of Medicine, Faculty of Medicine, Laval University, Québec, Canada

Correspondence and offprint requests to: Paul Isenring, Nephrology Division, Hôpital L’Hôtel-Dieu de Québec, 11 Côte du Palais, Québec (QC) G1R 2J6, Canada. Tel: +1-418-691-5151-(15477); Fax: +1-418-692-5795; E-mail: paul.isenring{at}crhdq.ulaval.ca



  Abstract

Background. Thrombotic thrombocytopenic purpura (TTP) and haemolytic uraemic syndrome (HUS) are thrombotic microangiopathies (TMAs). They are generally diagnosed and treated by plasmapheresis in the presence of non-immune haemolytic anaemia and thrombocytopenia. Yet, many individuals admitted in our hospital for athrombocytopenic renal failure of unknown cause were reported to have TMA as main lesion on kidney biopsies.

Methods. Speculating that this presentation is not uncommon and that the underlying lesion might not be suspected because of current diagnostic criteria, we determined its prevalence and other accompanying features through a retrospective single-centre cohort of 50 cases where TMA had been identified histologically.

Results. At presentation, normal serum platelets were common (44%) but still accompanied by abnormal serum LDH in most subjects. End-stage renal disease and mortality at 5 years were also high especially in the athrombocytopenic group, but unrelated to the underlying aetiology of TMA. Importantly, several subjects in both groups received and apparently responded to plasmapheresis.

Conclusion. In the absence of thrombocytopenia, TMA should still be contemplated when renal failure is associated with high serum LDH and its possible treatment with plasmapheresis assessed through prospective trials.

Keywords: haemolytic uraemic syndrome; plasmapheresis; renal failure; serum LDH; thrombotic microangiopathies

Received for publication: 11. 6.08
Accepted in revised form: 18.11.08


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