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

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...

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Autores principales: De Serres, Sacha A., Isenring, Paul
Formato: Texto
Lenguaje:English
Publicado: Oxford University Press 2009
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2644632/
https://www.ncbi.nlm.nih.gov/pubmed/19096082
http://dx.doi.org/10.1093/ndt/gfn687
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author De Serres, Sacha A.
Isenring, Paul
author_facet De Serres, Sacha A.
Isenring, Paul
author_sort De Serres, Sacha A.
collection PubMed
description 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.
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spelling pubmed-26446322009-02-25 Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria De Serres, Sacha A. Isenring, Paul Nephrol Dial Transplant Preliminary Communication 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. Oxford University Press 2009-03 2008-12-18 /pmc/articles/PMC2644632/ /pubmed/19096082 http://dx.doi.org/10.1093/ndt/gfn687 Text en © The Author [2008]. http://creativecommons.org/licenses/by-nc/2.0/uk/ 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
spellingShingle Preliminary Communication
De Serres, Sacha A.
Isenring, Paul
Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
title Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
title_full Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
title_fullStr Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
title_full_unstemmed Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
title_short Athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
title_sort athrombocytopenic thrombotic microangiopathy, a condition that could be overlooked based on current diagnostic criteria
topic Preliminary Communication
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2644632/
https://www.ncbi.nlm.nih.gov/pubmed/19096082
http://dx.doi.org/10.1093/ndt/gfn687
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