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hypereosinophilic syndrome

Ming Gao, Weihua Zhang, Waiou Zhao, Ling Qin, Fei Pei, Yang Zheng
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disease characterized by hypereosinophilia and its ensuing organ damage. Cardiac involvement is divided into 3 chronological stages: an acute necrotic stage; a thrombus formation stage; and a fibrotic stage. Infiltration of the myocardium by eosinophilic cells followed by endomyocardial fibrosis is known as "Loeffler endocarditis." PATIENT CONCERNS: We report a case of a 60-year-old man diagnosed with left-sided restrictive cardiomyopathy...
March 2018: Medicine (Baltimore)
Dissanayake Mudiyanselage Priyantha Udaya Kumara Ralapanawa, Kulatunga Wijekoon Mudiyanselage Pramitha Prabhashini Kumarihamy, Miriyalini Sundararajah, Widana Arachchilage Thilak Ananda Jayalath
BACKGROUND: Eosinophilic myocarditis is one of the fatal complications of idiopathic hypereosinophilic syndromes. Given the rarity of this form of myocarditis, it is often under-recognized. We describe a young girl who presented with features of heart failure. To our knowledge, this is the first reported case of eosinophilic myocarditis in a young Sri Lankan female. CASE PRESENTATION: A previously healthy 21 year old Sri Lankan female admitted with shortness of breath for 1 week duration with associated low grade fever and profuse sweating...
March 9, 2018: BMC Research Notes
Barbara A Brunet, Rebecca M Sugg, Patricia Stewart
No abstract text is available yet for this article.
February 28, 2018: Annals of Allergy, Asthma & Immunology
Merin Kuruvilla
Hypereosinophilic syndrome (HES) is a rare disease defined by organ damage directly attributable to hypereosinophilia that is either primary (neoplastic), secondary (reactive) or idiopathic. The mainstay of therapy in idiopathic HES centers around systemic steroids, and cytoreduction with hydroxyurea and interferon-alfa in steroid refractory cases. We describe the successful treatment of recalcitrant, idiopathic cutaneous HES with reslizumab, a humanized interleukin-5 blocker.
February 26, 2018: Annals of Allergy, Asthma & Immunology
Massimo Radin, Luca Bertero, Dario Roccatello, Savino Sciascia
The hypereosinophilic syndrome is a rare disease characterized by the association between high absolute eosinophil count and eosinophil-mediated organ damage. We describe a case of a 70-year-old male with an absolute eosinophil count of 2130 cells/µL. Clinical symptoms and signs included the following: severe asthenia, axonal sensitive motor neuropathy, basal pleural effusion with signs of hypoventilation on chest radiography, and gastrointestinal symptoms such as severe diarrhea, weight loss (-10 kg in 6 months), abdominal pain, and vomiting...
January 2018: Journal of Investigative Medicine High Impact Case Reports
Can Chen, Kuang Chen, Xilian Huang, Kaile Wang, Shenxian Qian
The current study presents the case of a 9-year-old Chinese boy who presented with eosinophilia and elevated serum levels of immunoglobulin G4 (IgG4). A bone marrow puncture identified an elevated eosinophil rate of 23% (normal range, <5%), which indicated eosinophilia. However, gene analysis, fluorescent in situ hybridization and other examinations, including bone marrow aspiration, blood routine, auto-antibody tests and parasitic and allergens screening, contradicted a diagnosis of secondary or clonal eosinophilia...
March 2018: Experimental and Therapeutic Medicine
Satoshi Kaito, Sakiko Harada, Masao Tsukada, Akira Kojima, Kanichi Iwama, Kazunari Yamada, Kouichi Kajiwara, Kentaro Morikawa, Keiki Shimizu, Yasuji Kozai, Hideki Koudo
A 35-year-old man who previously underwent splenectomy for hereditary spherocytosis at age 29 visited our hospital complaining of fatigue that had started 7 days ago and right upper abdominal pain. Laboratory data showed increased white blood cell and eosinophil count accompanied by severe transaminitis and clotting abnormalities. Computed tomography scan showed multiple embolisms in the portal vein, superior mesenteric vein, right pulmonary artery, and inferior vena cava. Severe liver damage presumably caused by portal vein thrombosis was also observed...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Christopher C Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau, Luke Y C Chen
No abstract text is available yet for this article.
February 2018: American Journal of the Medical Sciences
(no author information available yet)
No abstract text is available yet for this article.
January 1, 2018: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Attila Nemes, Imelda Marton, Péter Domsik, Anita Kalapos, Éva Pósfai, Szabolcs Modok, Zita Borbényi, Tamás Forster
Background: Persistent eosinophilia and eosinophil-mediated single- or multiple-organ damage are typical features of hypereosinophilic syndrome (HES). Theoretically, eosinophilic infiltration of the ascending aortic wall could not be excluded in HES, therefore the present study aimed to test whether HES is associated with abnormalities in aortic elastic properties. Methods: The present study comprised 10 HES patients (mean age: 57.6±10.1 years, 5 males) without known cardiovascular disease, their results were compared to 19 age-, gender- and risk factor-matched controls (59...
December 2017: Quantitative Imaging in Medicine and Surgery
Grzegorz Helbig
Hypereosinophilic syndromes (HES) encompass a group of disorders defined by sustained peripheral blood hypereosinophilia >1500/mm 3 and evidence of eosinophilia-associated organ impairment. Approximately 10% of HES patients may harbor a cryptic deletion on chromosome 4 leading to formation of the FIP1L1-PDGFRA (F/P) fusion gene; these patients are diagnosed as F/P-mutated myeloid/lymphoid neoplasms with eosinophilia (MLN-eo). Areas covered: This review discusses the results of IM treatment in HES depending on mutation status...
February 2018: Expert Review of Clinical Immunology
Jean Emmanuel Kahn, Matthieu Groh, Guillaume Lefèvre
Hypereosinophilia (HE) is a heterogeneous condition that can be reported in various (namely inflammatory, allergic, infectious, or neoplastic) diseases with distinct pathophysiological pathways. In 1975, Chusid et al. published the first diagnostic criteria of hypereosinophilic syndromes (HES). Over the years, as both basic and clinical knowledge improved, several updates have been suggested, with a focus on better distinguishing isolated or asymptomatic eosinophilia from diseases with specific eosinophil-related organ damage...
2017: Frontiers in Medicine
Kristin Eden, Daniel E Rothschild, Dylan K McDaniel, Bettina Heid, Irving C Allen
Eosinophilic esophagitis (EoE) is an allergic disease of the esophagus driven by T cell and eosinophil responses to dietary allergens, resulting in chronic mucosal inflammation. Few spontaneous animal models of esophageal eosinophilia exist, with most studies relying on artificial sensitization procedures. NF-κB-inducing kinase (NIK; MAP3K14) is a key signaling molecule of the noncanonical NF-κB (NFKB1) pathway, an alternative signaling cascade producing chemokines involved in lymphoid stroma development and leukocyte trafficking...
December 19, 2017: Disease Models & Mechanisms
A Nemes, I Marton, P Domsik, A Kalapos, É Pósfai, S Modok, Á Kormányos, N Ambrus, Z Borbényi, T Forster
BACKGROUND: Idiopathic hypereosinophilic syndrome is characterized by a persistent eosinophil blood count of >1.5 × 109 cells/l and organ damage, independent of the primary and secondary causes of eosinophilia. The purpose of the present study was to assess the three-dimensional speckle tracking echocardiography-derived right atrial volumetric and functional properties between hypereosinophilic syndrome patients and matched controls. METHODS: A total of 11 patients with idiopathic hypereosinophilic syndrome and 22 age- and gender-matched healthy controls were enrolled in the study...
December 12, 2017: Herz
Christopher C Cheung, Maggie Constantine, Amir Ahmadi, Carolyn Shiau, Luke Y C Chen
Persistent eosinophilia can cause cardiac tissue damage, typically in the form of eosinophilic myocarditis, whether the underlying cause is reactive, a clonal myeloid disorder, or idiopathic hypereosinophilic syndrome (HES). Eosinophilic myocarditis ranges from mild localized disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications and endomyocardial fibrosis. Systemic treatment varies widely depending on the underlying cause, so thorough investigation and precise diagnosis are essential...
November 2017: American Journal of the Medical Sciences
Xuanyi Jin, Chunyan Ma, Yonghuai Wang, Jun Yang
Loeffler endocarditis is a rare comprehensive cardiac manifestation caused by eosinophilic cell infiltrations and is present in 50%-60% of patients with hypereosinophilic syndrome (HES). Left ventricle (LV) endocardial systolic dysfunction is a major cause of morbidity and mortality in HES and Loeffler endocarditis. We present a case of Loeffler endocarditis, whose left ventricular (LV) systolic dysfunction and endocardial systolic dysfunction were first neglected by conventional transthoracic echocardiography (TTE), but were later pointed out by layer-specific longitudinal strain analysis...
November 17, 2017: International Heart Journal
Fei Li Kuang, Amy D Klion
Hypereosinophilic syndromes (HES) are a heterogeneous group of rare disorders defined by the presence of marked peripheral or tissue eosinophilia resulting in end-organ damage. Although conventional therapies, including glucocorticoids, hydroxyurea, and IFN-α, are initially effective in reducing eosinophilia and symptoms in a majority of patients with platelet-derived growth factor mutation-negative HES, the development of resistance and treatment-related toxicity are common. In contrast, targeted therapy with the tyrosine kinase inhibitor, imatinib, is well tolerated but effective only in the subset of patients with HES with a primary myeloid disorder...
November 2017: Journal of Allergy and Clinical Immunology in Practice
N Dong, X Y Dong
Objective: To explore clinical features of idiopathic hypereosinophilic syndrome combined with pulmonary embolism. Method: A retrospective analysis of a patient with idiopathic hypereosinophilic syndrome and pulmonary embolism diagnosed and treated in the Respiratory Department of Shanghai Children's Hospital in September 2016 was performed. A literature search was performed with"Eosinophils increased, thrombosis"as the Chinese keywords in Wanfang database and"idiopathic hypereosinophilic syndrome, deep vein thrombosis"as the English key words in PubMed database...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Jason Gotlib
DISEASE OVERVIEW: The eosinophilias encompass a broad range of nonhematologic (secondary or reactive) and hematologic (primary, clonal) disorders with potential for end-organ damage. DIAGNOSIS: Hypereosinophilia has generally been defined as a peripheral blood eosinophil count greater than 1500/mm3 and may be associated with tissue damage. After exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on a combination of morphologic review of the blood and marrow, standard cytogenetics, fluorescent in situ-hybridization, flow immunocytometry, and T-cell clonality assessment to detect histopathologic or clonal evidence for an acute or chronic myeloid or lymphoproliferative disorder...
November 2017: American Journal of Hematology
Carsten Schwarz, Thilo Müller, Susanne Lau, Kirn Parasher, Doris Staab, Ulrich Wahn
BACKGROUND: Mepolizumab was originally intended as a therapeutic agent for atopic asthma in adults, and consequently, little is known about its use in children. Up to now, corticosteroids have formed the basis of the initial treatment of hypereosinophilic syndromes and are shown to be effective in most patients. To analyze the effect of mepolizumab in children is the aim of this study. METHODS: We are reporting the experience of the effect of mepolizumab in 2 pediatric patients with hypereosinophilic syndrome that was not sufficiently controlled by other drugs...
February 2018: Pediatric Allergy and Immunology
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