Hypereosinophilic syndrome presenting as coagulopathy

Abstract 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, p...

Full description

Bibliographic Details
Main Authors: Kestutis Aukstuolis, Jocelyn J. Cooper, Katherine Altman, Anna Lang, Andrew G. Ayars
Format: Article
Language:English
Published: BMC 2022-03-01
Series:Allergy, Asthma & Clinical Immunology
Subjects:
Online Access:https://doi.org/10.1186/s13223-022-00666-2
_version_ 1818358041822625792
author Kestutis Aukstuolis
Jocelyn J. Cooper
Katherine Altman
Anna Lang
Andrew G. Ayars
author_facet Kestutis Aukstuolis
Jocelyn J. Cooper
Katherine Altman
Anna Lang
Andrew G. Ayars
author_sort Kestutis Aukstuolis
collection DOAJ
description Abstract 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.
first_indexed 2024-12-13T20:22:42Z
format Article
id doaj.art-73b0a379430a4604acae1ae96e0e6d8b
institution Directory Open Access Journal
issn 1710-1492
language English
last_indexed 2024-12-13T20:22:42Z
publishDate 2022-03-01
publisher BMC
record_format Article
series Allergy, Asthma & Clinical Immunology
spelling doaj.art-73b0a379430a4604acae1ae96e0e6d8b2022-12-21T23:32:38ZengBMCAllergy, Asthma & Clinical Immunology1710-14922022-03-011811510.1186/s13223-022-00666-2Hypereosinophilic syndrome presenting as coagulopathyKestutis Aukstuolis0Jocelyn J. Cooper1Katherine Altman2Anna Lang3Andrew G. Ayars4Division of Allergy and Infectious Diseases, Department of Medicine, University of WashingtonDivision of Internal Medicine, Department of Medicine, University of WashingtonDivision of Allergy and Infectious Diseases, Department of Medicine, University of WashingtonDivision of Allergy and Infectious Diseases, Department of Medicine, University of WashingtonDivision of Allergy and Infectious Diseases, Department of Medicine, University of WashingtonAbstract 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.https://doi.org/10.1186/s13223-022-00666-2Hypereosinophilic syndromeThrombosisBowel perforationAnti-IL-5-receptor therapyBenralizumabIschemic colitis
spellingShingle Kestutis Aukstuolis
Jocelyn J. Cooper
Katherine Altman
Anna Lang
Andrew G. Ayars
Hypereosinophilic syndrome presenting as coagulopathy
Allergy, Asthma & Clinical Immunology
Hypereosinophilic syndrome
Thrombosis
Bowel perforation
Anti-IL-5-receptor therapy
Benralizumab
Ischemic colitis
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 Hypereosinophilic syndrome
Thrombosis
Bowel perforation
Anti-IL-5-receptor therapy
Benralizumab
Ischemic colitis
url https://doi.org/10.1186/s13223-022-00666-2
work_keys_str_mv AT kestutisaukstuolis hypereosinophilicsyndromepresentingascoagulopathy
AT jocelynjcooper hypereosinophilicsyndromepresentingascoagulopathy
AT katherinealtman hypereosinophilicsyndromepresentingascoagulopathy
AT annalang hypereosinophilicsyndromepresentingascoagulopathy
AT andrewgayars hypereosinophilicsyndromepresentingascoagulopathy