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      Eosinophilic myocarditis and hypereosinophilic syndrome

      brief-report

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          Abstract

          Hypereosinophilic syndrome (HES) is a heterogeneous group of hematological disorders characterized by a chronic, unexplained hypereosinophilia with tissue damage. Cardiac involvement occurs in ∼20% of patients with HES and represents a major turning point. Cardiac injuries related to eosinophilia are divided into three chronological phases: eosinophilic infiltration, thrombosis, and fibrosis. We report a case of a 33-year-old woman diagnosed with HES, with pulmonary and gastrointestinal involvement and eosinophilic myocarditis in cardiogenic shock. The evolution was favorable with dobutamine, anticoagulation, corticosteroids, and later, β-blockers and angiotensin-converting enzyme inhibitors. Cardiac involvement in HES is rare but carries a poor prognosis. Corticosteroids are considered by many to be the mainstay of treatment. Although new treatments have been suggested, only a few seem promising.

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          Most cited references9

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          Hypereosinophilic syndrome: a multicenter, retrospective analysis of clinical characteristics and response to therapy.

          Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders defined by persistent blood eosinophilia > or =1.5 x 10(9)/L, absence of a secondary cause, and evidence of eosinophil-associated pathology. With the exception of a recent multicenter trial of mepolizumab (anti-IL-5 mAb), published therapeutic experience has been restricted to case reports and small case series. The purpose of the study was to collect and summarize baseline demographic, clinical, and laboratory characteristics in a large, diverse cohort of patients with HES and to review responses to treatment with conventional and novel therapies. Clinical and laboratory data from 188 patients with HES, seen between January 2001 and December 2006 at 11 institutions in the United States and Europe, were collected retrospectively by chart review. Eighteen of 161 patients (11%) tested were Fip1-like 1-platelet-derived growth factor receptor alpha (FIP1L1-PDGFRA) mutation-positive, and 29 of 168 patients tested (17%) had a demonstrable aberrant or clonal T-cell population. Corticosteroid monotherapy induced complete or partial responses at 1 month in 85% (120/141) of patients with most remaining on maintenance doses (median, 10 mg prednisone equivalent daily for 2 months to 20 years). Hydroxyurea and IFN-alpha (used in 64 and 46 patients, respectively) were also effective, but their use was limited by toxicity. Imatinib (used in 68 patients) was more effective in patients with the FIP1L1-PDGFRA mutation (88%) than in those without (23%; P < .001). This study, the largest clinical analysis of patients with HES to date, not only provides useful information for clinicians but also should stimulate prospective trials to optimize treatment of HES.
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            The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.

            Several closely related disease entities make up the idiopathic hypereosinophilic syndrome (HES). The syndrome is manifest by persistent and prolonged eosinophilia with organ damage. A group of 14 patients had hematologic, cardiac, and neurologic abnormalities attributable to this disease. Patient survival and response to chemotherapy was significantly better in this group than in previously reported patients. The etiology of HES remains unknown, as does the mechanism of tissue damage.
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              Current Diagnostic and Therapeutic Aspects of Eosinophilic Myocarditis

              Eosinophilic myocarditis (EM) represents a rare form of myocardial inflammation with very heterogeneous aetiology. In developed countries, the most prevalent causes of EM are hypersensitivity or allergic reactions, as well as hematological diseases leading to eosinophilia. The disease may have a variable clinical presentation, ranging from asymptomatic forms to life-threatening conditions. Most patients with EM have marked eosinophilia in peripheral blood. Endomyocardial biopsy needs to be performed in most cases in order to establish a definitive diagnosis of EM. The therapy depends on the underlying aetiology. Immunosuppressive therapy represents the treatment mainstay in the majority of EM forms.
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                Author and article information

                Contributors
                Journal
                J Saudi Heart Assoc
                J Saudi Heart Assoc
                Journal of the Saudi Heart Association
                Elsevier
                1016-7315
                2212-5043
                17 November 2016
                July 2017
                17 November 2016
                : 29
                : 3
                : 211-213
                Affiliations
                [a ]Department of Cardiology, University Hospital of Mohammed VI, Oujda, Morocco aMorocco
                Author notes
                [* ]Corresponding author at: Department of Cardiology, University Hospital of Mohammed VI, BP 4806, Oujda University, 60049 Oujda, Morocco.Department of CardiologyUniversity Hospital of Mohammed VIBP 4806, Oujda University60049 OujdaMorocco hanane0802@ 123456gmail.com
                Article
                S1016-7315(16)30166-X
                10.1016/j.jsha.2016.11.001
                5475350
                28652675
                121151f6-5169-4378-914f-67e35cda4efe
                © 2016 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 28 August 2016
                : 10 November 2016
                Categories
                Case Report

                cardiogenic shock,corticosteroids,eosinophilic myocarditis,hypereosinophilic syndrome,imatinib

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