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      Idiopathic Hypereosinophilic Syndrome in an Elderly Female: A Case Report

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          Abstract

          Patient: Female, 83

          Final Diagnosis: Idiopathic hypereosinophilic syndrome

          Symptoms: Dyspnea

          Medication: —

          Clinical Procedure: —

          Specialty: Allergology

          Objective:

          Rare disease

          Background:

          Hypereosinophilic syndrome (HES) is defined as hypereosinophilia with eosinophil mediated organ damage or dysfunction, provided that other causes of organ damage have been excluded.

          Case Report:

          An 83-year-old female presented with worsening dyspnea for 3 weeks. She was initially diagnosed with bronchitis and prescribed oral antibiotics along with prednisone taper. However, her dyspnea continued to worsen requiring hospitalization. Physical examination was significant for signs of volume overload. Laboratory investigations were notable for leukocytosis with eosinophilia, elevated BNP (brain natriuretic peptide) and troponin. Electrocardiogram (ECG) showed normal sinus rhythm with non-specific ST-T wave changes. Computed tomography (CT) scan of the chest showed pulmonary edema, bilateral peripheral ground glass opacities, and pleural effusions. Transthoracic echocardiogram (TTE) revealed an ejection fraction (EF) of 45%. She was diagnosed with NSTEMI (non-ST-elevation myocardial infarction) with new onset heart failure; appropriate management was initiated. Left heart catheterization did not show any significant obstructive lesions. Presence of peripheral ground glass opacities on the CT chest scan and eosinophilia raised suspicion for HES. Thorough HES workup was done, all tests came back negative except for elevated serum IgE level. Cardiac biopsy returned positive for eosinophilic myocarditis. Bone marrow biopsy showed 20% eosinophils. Positron emission tomography (PET) scan did not show any hypermetabolic lesions to suggest malignancy. The patient was managed for idiopathic HES with high dose steroids resulting in significant clinical improvement.

          Conclusions:

          About 40% of patient with HES manifest cardiac involvement, and one quarter of patients with HES have pulmonary involvement with variable radiologic findings. Steroids remain the mainstay treatment for idiopathic HES.

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          Most cited references9

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          Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes.

          Eosinophilia is an important indicator of various neoplastic and nonneoplastic conditions. Depending on the underlying disease and mechanisms, eosinophil infiltration can lead to organ dysfunction, clinical symptoms, or both. During the past 2 decades, several different classifications of eosinophilic disorders and related syndromes have been proposed in various fields of medicine. Although criteria and definitions are, in part, overlapping, no global consensus has been presented to date. The Year 2011 Working Conference on Eosinophil Disorders and Syndromes was organized to update and refine the criteria and definitions for eosinophilic disorders and to merge prior classifications in a contemporary multidisciplinary schema. A panel of experts from the fields of immunology, allergy, hematology, and pathology contributed to this project. The expert group agreed on unifying terminologies and criteria and a classification that delineates various forms of hypereosinophilia, including primary and secondary variants based on specific hematologic and immunologic conditions, and various forms of the hypereosinophilic syndrome. For patients in whom no underlying disease or hypereosinophilic syndrome is found, the term hypereosinophilia of undetermined significance is introduced. The proposed novel criteria, definitions, and terminologies should assist in daily practice, as well as in the preparation and conduct of clinical trials. Copyright © 2012 American Academy of Allergy, Asthma & Immunology. Published by Mosby, Inc. All rights reserved.
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            • Record: found
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            Incidence of myeloproliferative hypereosinophilic syndrome in the United States and an estimate of all hypereosinophilic syndrome incidence.

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              NIH conference. The idiopathic hypereosinophilic syndrome. Clinical, pathophysiologic, and therapeutic considerations.

              The idiopathic hypereosinophilic syndrome (HES) represents a heterogeneous group of disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. Fifty patients with the idiopathic HES were studied over 11 years of the National Institutes of Health. Multiple organ systems were involved; bone marrow hypereosinophilia was common to all patients, but the most severe clinicopathologic involvement was of the heart and nervous system. Postmortem gross pathologic examination of the hearts of patients with idiopathic and nonidiopathic HES suggested that the common mechanism of cardiac disease is the eosinophilia. Endomyocardial biopsy findings showed that the endothelial cells in the endocardium and of the microvasculature were the primary targets of the tissue damage. This damage initiates thrombosis; endocardial fibrosis and restrictive endomyocardopathy may follow. In-vitro culture of circulating eosinophil colony-forming units showed some normal studies, some studies showing increased progenitor cells committed to eosinophil development, and others showing an excess production of eosinophil colony-stimulating factor. Chemotherapy to lower the eosinophil counts has resulted in marked improvement of HES prognosis, as have agressive medical and surgical approaches to cardiovascular complications.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2019
                23 March 2019
                : 20
                : 381-384
                Affiliations
                [1 ]Department of Internal Medicine, University Hospital Cleveland Medical Center/Case Western Reserve University, Cleveland, OH, U.S.A.
                [2 ]Department of Pulmonary, Critical Care and Sleep Medicine, University of Colorado, Denver, CO, U.S.A.
                Author notes

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Conflict of interest: None declared

                Corresponding Author: Faiza Khalid, e-mail: faizakhalid24@ 123456gmail.com , Faiza.Khalid@ 123456UHhospitals.org
                Article
                912747
                10.12659/AJCR.912747
                6441311
                30902963
                9079964d-a7c1-42db-bc76-8a0dd8758d0c
                © Am J Case Rep, 2019

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 14 August 2018
                : 06 December 2018
                Categories
                Articles

                disease management,hypereosinophilic syndrome,myocarditis

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