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Hypereosinophilic syndrome presenting as coagulopathy
BACKGROUND: Hypereosinophilic syndrome (HES) is an extremely uncommon group of disorders. It rarely presents with coagulopathy without cardiac involvement. CASE PRESENTATION: A 33-year-old previously healthy male with no history of atopic disease presented with abdominal pain, hematochezia, peripher...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8941788/ https://www.ncbi.nlm.nih.gov/pubmed/35317854 http://dx.doi.org/10.1186/s13223-022-00666-2 |
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author | Aukstuolis, Kestutis Cooper, Jocelyn J. Altman, Katherine Lang, Anna Ayars, Andrew G. |
author_facet | Aukstuolis, Kestutis Cooper, Jocelyn J. Altman, Katherine Lang, Anna Ayars, Andrew G. |
author_sort | Aukstuolis, Kestutis |
collection | PubMed |
description | BACKGROUND: Hypereosinophilic syndrome (HES) is an extremely uncommon group of disorders. It rarely presents with coagulopathy without cardiac involvement. CASE PRESENTATION: A 33-year-old previously healthy male with no history of atopic disease presented with abdominal pain, hematochezia, peripheral eosinophilia as high as 10,000 eos/µL, right and left portal vein, mesenteric, and splenic vein thrombi with ischemic colitis resulting in hemicolectomy and small bowel resection. Despite an extensive workup for primary and secondary etiologies of hypereosinophilia by hematology/oncology, infectious disease, rheumatology and allergy/immunology, no other clear causes were identified, and the patient was diagnosed with idiopathic HES. His eosinophilia was successfully treated with high-dose oral corticosteroids (OCS) and subsequently transitioned to anti-IL-5-receptor therapy with benralizumab. He has continued this treatment for over a year with no recurrence of eosinophilia or thrombosis while on benralizumab. CONCLUSION: In patients with an unexplained coagulopathy and eosinophilia, eosinophilic disorders such as HES should be considered. Corticosteroid-sparing agents, such as benralizumab show promise for successfully treating these patients. |
format | Online Article Text |
id | pubmed-8941788 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-89417882022-03-24 Hypereosinophilic syndrome presenting as coagulopathy Aukstuolis, Kestutis Cooper, Jocelyn J. Altman, Katherine Lang, Anna Ayars, Andrew G. Allergy Asthma Clin Immunol Case Report BACKGROUND: Hypereosinophilic syndrome (HES) is an extremely uncommon group of disorders. It rarely presents with coagulopathy without cardiac involvement. CASE PRESENTATION: A 33-year-old previously healthy male with no history of atopic disease presented with abdominal pain, hematochezia, peripheral eosinophilia as high as 10,000 eos/µL, right and left portal vein, mesenteric, and splenic vein thrombi with ischemic colitis resulting in hemicolectomy and small bowel resection. Despite an extensive workup for primary and secondary etiologies of hypereosinophilia by hematology/oncology, infectious disease, rheumatology and allergy/immunology, no other clear causes were identified, and the patient was diagnosed with idiopathic HES. His eosinophilia was successfully treated with high-dose oral corticosteroids (OCS) and subsequently transitioned to anti-IL-5-receptor therapy with benralizumab. He has continued this treatment for over a year with no recurrence of eosinophilia or thrombosis while on benralizumab. CONCLUSION: In patients with an unexplained coagulopathy and eosinophilia, eosinophilic disorders such as HES should be considered. Corticosteroid-sparing agents, such as benralizumab show promise for successfully treating these patients. BioMed Central 2022-03-22 /pmc/articles/PMC8941788/ /pubmed/35317854 http://dx.doi.org/10.1186/s13223-022-00666-2 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Case Report Aukstuolis, Kestutis Cooper, Jocelyn J. Altman, Katherine Lang, Anna Ayars, Andrew G. Hypereosinophilic syndrome presenting as coagulopathy |
title | Hypereosinophilic syndrome presenting as coagulopathy |
title_full | Hypereosinophilic syndrome presenting as coagulopathy |
title_fullStr | Hypereosinophilic syndrome presenting as coagulopathy |
title_full_unstemmed | Hypereosinophilic syndrome presenting as coagulopathy |
title_short | Hypereosinophilic syndrome presenting as coagulopathy |
title_sort | hypereosinophilic syndrome presenting as coagulopathy |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8941788/ https://www.ncbi.nlm.nih.gov/pubmed/35317854 http://dx.doi.org/10.1186/s13223-022-00666-2 |
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