Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Al-Shaghana, May, Bentall, Andrew, Jesky, Mark D., Lester, William, Lipkin, Graham
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2017
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
_version_ 1783243979841601536
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
work_keys_str_mv AT alshaghanamay earlyeculizumabuseinatypicalhaemolyticuraemicsyndromeinajehovahswitnessrefusingbloodproducts
AT bentallandrew earlyeculizumabuseinatypicalhaemolyticuraemicsyndromeinajehovahswitnessrefusingbloodproducts
AT jeskymarkd earlyeculizumabuseinatypicalhaemolyticuraemicsyndromeinajehovahswitnessrefusingbloodproducts
AT lesterwilliam earlyeculizumabuseinatypicalhaemolyticuraemicsyndromeinajehovahswitnessrefusingbloodproducts
AT lipkingraham earlyeculizumabuseinatypicalhaemolyticuraemicsyndromeinajehovahswitnessrefusingbloodproducts