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Atypical hemolytic uremic syndrome after myomectomy: A case report

Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy due to inability to regulate the complement cascade, resulting in thrombocytopenia, intravascular hemolysis, and end-organ damage. Over 70% of cases are associated with mutations in complement or complement regula...

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Autores principales: Musselman, Kelsey, Laurence, Jeffrey, Magro, Cynthia, Rahbari, Pasha, Di Vitantonio, Thomas, Havryliuk, Yelena
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9234065/
https://www.ncbi.nlm.nih.gov/pubmed/35769946
http://dx.doi.org/10.1016/j.crwh.2022.e00424
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author Musselman, Kelsey
Laurence, Jeffrey
Magro, Cynthia
Rahbari, Pasha
Di Vitantonio, Thomas
Havryliuk, Yelena
author_facet Musselman, Kelsey
Laurence, Jeffrey
Magro, Cynthia
Rahbari, Pasha
Di Vitantonio, Thomas
Havryliuk, Yelena
author_sort Musselman, Kelsey
collection PubMed
description Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy due to inability to regulate the complement cascade, resulting in thrombocytopenia, intravascular hemolysis, and end-organ damage. Over 70% of cases are associated with mutations in complement or complement regulatory proteins, and some two-thirds have recognized complement-activating conditions triggering an aHUS event. We describe a case of aHUS after abdominal myomectomy in a 42-year-old woman that was managed with plasma exchange and eculizumab (an anti-C5 monoclonal antibody). The diagnosis was confirmed by biopsy of normal-appearing deltoid skin that demonstrated microvascular C5b-9 deposition, diagnostic of systemic complement pathway activation. Although extremely uncommon following gynecologic surgery, aHUS should be considered in the setting of postoperative oliguric acute kidney injury, as prompt diagnosis is necessary to prevent significant morbidity and mortality.
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spelling pubmed-92340652022-06-28 Atypical hemolytic uremic syndrome after myomectomy: A case report Musselman, Kelsey Laurence, Jeffrey Magro, Cynthia Rahbari, Pasha Di Vitantonio, Thomas Havryliuk, Yelena Case Rep Womens Health Article Atypical hemolytic uremic syndrome (aHUS) is a rare form of thrombotic microangiopathy due to inability to regulate the complement cascade, resulting in thrombocytopenia, intravascular hemolysis, and end-organ damage. Over 70% of cases are associated with mutations in complement or complement regulatory proteins, and some two-thirds have recognized complement-activating conditions triggering an aHUS event. We describe a case of aHUS after abdominal myomectomy in a 42-year-old woman that was managed with plasma exchange and eculizumab (an anti-C5 monoclonal antibody). The diagnosis was confirmed by biopsy of normal-appearing deltoid skin that demonstrated microvascular C5b-9 deposition, diagnostic of systemic complement pathway activation. Although extremely uncommon following gynecologic surgery, aHUS should be considered in the setting of postoperative oliguric acute kidney injury, as prompt diagnosis is necessary to prevent significant morbidity and mortality. Elsevier 2022-06-11 /pmc/articles/PMC9234065/ /pubmed/35769946 http://dx.doi.org/10.1016/j.crwh.2022.e00424 Text en © 2022 The Authors https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Article
Musselman, Kelsey
Laurence, Jeffrey
Magro, Cynthia
Rahbari, Pasha
Di Vitantonio, Thomas
Havryliuk, Yelena
Atypical hemolytic uremic syndrome after myomectomy: A case report
title Atypical hemolytic uremic syndrome after myomectomy: A case report
title_full Atypical hemolytic uremic syndrome after myomectomy: A case report
title_fullStr Atypical hemolytic uremic syndrome after myomectomy: A case report
title_full_unstemmed Atypical hemolytic uremic syndrome after myomectomy: A case report
title_short Atypical hemolytic uremic syndrome after myomectomy: A case report
title_sort atypical hemolytic uremic syndrome after myomectomy: a case report
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9234065/
https://www.ncbi.nlm.nih.gov/pubmed/35769946
http://dx.doi.org/10.1016/j.crwh.2022.e00424
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