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Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products
Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471596/ https://www.ncbi.nlm.nih.gov/pubmed/28638625 http://dx.doi.org/10.1093/omcr/omx025 |
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author | Al-Shaghana, May Bentall, Andrew Jesky, Mark D. Lester, William Lipkin, Graham |
author_facet | Al-Shaghana, May Bentall, Andrew Jesky, Mark D. Lester, William Lipkin, Graham |
author_sort | Al-Shaghana, May |
collection | PubMed |
description | Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah’s Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS. Despite a life threatening illness, the patient declined the use of blood components and plasma exchange. Eculizumab was administered early with subsequent improvement and resolution of haemolysis, return to baseline renal function whilst avoiding use of blood products. This case demonstrates the effective use of eculizumab for life saving therapy in a patient refusing blood products. It highlights the importance of accessibility for high cost therapies, but the disparity in access between healthcare systems. |
format | Online Article Text |
id | pubmed-5471596 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Oxford University Press |
record_format | MEDLINE/PubMed |
spelling | pubmed-54715962017-06-21 Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products Al-Shaghana, May Bentall, Andrew Jesky, Mark D. Lester, William Lipkin, Graham Oxf Med Case Reports Case Report Thrombotic microangiopathy (TMA) is characterized by microscopic angiopathic haemolytic anaemia, thrombocytopenia and organ injury. Supportive therapies include the use of blood products. Recently the terminal complement inhibitor eculizumab has been approved in atypical haemolytic uraemic syndrome (aHUS) in some countries. We report the case of a 23-year-old female Jehovah’s Witness presenting with vaginal haemorrhage from thrombocytopaenia, severe anaemia (nadir Hb 28 g/L) and anuric acute kidney injury with TMA secondary to aHUS. Despite a life threatening illness, the patient declined the use of blood components and plasma exchange. Eculizumab was administered early with subsequent improvement and resolution of haemolysis, return to baseline renal function whilst avoiding use of blood products. This case demonstrates the effective use of eculizumab for life saving therapy in a patient refusing blood products. It highlights the importance of accessibility for high cost therapies, but the disparity in access between healthcare systems. Oxford University Press 2017-06-15 /pmc/articles/PMC5471596/ /pubmed/28638625 http://dx.doi.org/10.1093/omcr/omx025 Text en © The Author 2017. Published by Oxford University Press. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com |
spellingShingle | Case Report Al-Shaghana, May Bentall, Andrew Jesky, Mark D. Lester, William Lipkin, Graham Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products |
title | Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products |
title_full | Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products |
title_fullStr | Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products |
title_full_unstemmed | Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products |
title_short | Early eculizumab use in atypical haemolytic uraemic syndrome in a Jehovah’s Witness refusing blood products |
title_sort | early eculizumab use in atypical haemolytic uraemic syndrome in a jehovah’s witness refusing blood products |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5471596/ https://www.ncbi.nlm.nih.gov/pubmed/28638625 http://dx.doi.org/10.1093/omcr/omx025 |
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