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Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria

Paroxysmal nocturnal haemoglobinura is an acquired life‐threatening haemolytic condition, which is generally well controlled with terminal complement blockade with eculizumab. Whilst almost all patients treated with terminal complement inhibitors develop extravascular haemolysis, only a small propor...

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Detalles Bibliográficos
Autores principales: Davis, Amanda K., Bingham, Nicholas, Szer, Jeff
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435701/
https://www.ncbi.nlm.nih.gov/pubmed/37601872
http://dx.doi.org/10.1002/jha2.714
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author Davis, Amanda K.
Bingham, Nicholas
Szer, Jeff
author_facet Davis, Amanda K.
Bingham, Nicholas
Szer, Jeff
author_sort Davis, Amanda K.
collection PubMed
description Paroxysmal nocturnal haemoglobinura is an acquired life‐threatening haemolytic condition, which is generally well controlled with terminal complement blockade with eculizumab. Whilst almost all patients treated with terminal complement inhibitors develop extravascular haemolysis, only a small proportion of these results in symptomatic anaemia limiting their activities and requiring red cell transfusion. This case highlights the potential role for the C3 inhibitor, pegcetacoplan, in controlling both intravascular and extravascular haemolysis, and is the first case to report on the use of additional doses of pegcetacoplan to control breakthrough haemolysis.
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spelling pubmed-104357012023-08-19 Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria Davis, Amanda K. Bingham, Nicholas Szer, Jeff EJHaem Short Reports Paroxysmal nocturnal haemoglobinura is an acquired life‐threatening haemolytic condition, which is generally well controlled with terminal complement blockade with eculizumab. Whilst almost all patients treated with terminal complement inhibitors develop extravascular haemolysis, only a small proportion of these results in symptomatic anaemia limiting their activities and requiring red cell transfusion. This case highlights the potential role for the C3 inhibitor, pegcetacoplan, in controlling both intravascular and extravascular haemolysis, and is the first case to report on the use of additional doses of pegcetacoplan to control breakthrough haemolysis. John Wiley and Sons Inc. 2023-05-23 /pmc/articles/PMC10435701/ /pubmed/37601872 http://dx.doi.org/10.1002/jha2.714 Text en © 2023 The Authors. eJHaem published by British Society for Haematology and John Wiley & Sons Ltd. https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Short Reports
Davis, Amanda K.
Bingham, Nicholas
Szer, Jeff
Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria
title Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria
title_full Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria
title_fullStr Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria
title_full_unstemmed Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria
title_short Normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria
title_sort normalisation of haemoglobin and control of breakthrough haemolysis with increased frequency pegcetacoplan dosing in treated paroxysmal nocturnal haemoglobinuria
topic Short Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10435701/
https://www.ncbi.nlm.nih.gov/pubmed/37601872
http://dx.doi.org/10.1002/jha2.714
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