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hypereosinophilic syndrome

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https://www.readbyqxmd.com/read/29151479/a-case-of-loeffler-endocarditis-that-showed-endomyocardial-systolic-dysfunction-detected-by-layer-specific-strain-analysis
#1
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
https://www.readbyqxmd.com/read/29122152/biologic-agents-for-the-treatment-of-hypereosinophilic-syndromes
#2
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
https://www.readbyqxmd.com/read/29050117/-pediatric-idiopathic-hypereosinophilic-syndrome-with-pulmonary-embolism-a-case-report-and-review-of-literature
#3
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
https://www.readbyqxmd.com/read/29044676/world-health-organization-defined-eosinophilic-disorders-2017-update-on-diagnosis-risk-stratification-and-management
#4
REVIEW
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/mm(3) 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
https://www.readbyqxmd.com/read/28986919/mepolizumab-a-novel-option-for-the-treatment-of-hypereosinophilic-syndrome-in-childhood
#5
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 analyse the effect of mepolizumab in children is the aim of this study. METHODS: We are reporting the experience of the effect of mepolizumab in two paediatric patients of hypereosinophilic syndrome that was not sufficiently controlled by other drugs...
October 6, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28945486/hypereosinophilic-syndrome-with-central-nervous-system-involvement-two-case-reports-and-literature-review
#6
Hao Chen, Hafiz Khuram Raza, Jia Jing, Dayong Shen, Peng Xu, Su Zhou, Jie Zu, Xinxin Yang, Wei Zhang, Shenyang Zhang, Fang Hua, Guiyun Cui
OBJECTIVE: To report two cases of hypereosinophilic syndrome (HES) with central nervous system involvement and explore its possible pathogenesis. METHODS: We have analysed the clinical data and relevant features of two patients who presented themselves to The Affiliated Hospital of Xuzhou Medical University between 2012 and 2015. We have reviewed the relevant literature, elaborated the possible pathogenesis, and discussed the treatment options. RESULTS: Both patients had consistently high levels of absolute eosinophil count which led to multiple cerebral infarcts in the arterial border zone and small-vessel disease...
September 25, 2017: Brain Injury: [BI]
https://www.readbyqxmd.com/read/28890659/loeffler-endocarditis-a-unique-presentation-of-right-sided-heart-failure-due-to-eosinophil-induced-endomyocardial-fibrosis
#7
Amit Alam, Shankar Thampi, Shahryar G Saba, Rita Jermyn
Loeffler endocarditis is a rare restrictive cardiomyopathy caused by abnormal endomyocardial infiltration of eosinophils, with subsequent tissue damage from degranulation, eventually leading to fibrosis. Although an uncommon entity, it is still a disease with significant morbidity and mortality. Often identified only at late stages, treatment options are limited once fibrosis occurs, usually requiring heart failure medications or surgical intervention. We present a unique case of a woman with remote history of hypereosinophilic syndrome, attributed to treatment of rheumatoid arthritis with infliximab, who presented with symptoms of heart failure refractory to medical management and was found to have Loeffler endocarditis...
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/28885361/angioimmunoblastic-t-cell-lymphoma-and-hypereosinophilic-syndrome-with-fip1l1-pdgfra-fusion-gene-effectively-treated-with-imatinib-a-case-report
#8
Masayo Yamamoto, Katsuya Ikuta, Yasumichi Toki, Mayumi Hatayama, Motohiro Shindo, Yoshihiro Torimoto, Toshikatsu Okumura
RATIONALE: Hypereosinophilic syndrome (HES) is a rare disorder characterized by hypereosinophilia and organ damage. Some cases of HES are caused by the FIP1L1/PDGFRA fusion gene and respond to imatinib. FIP1L1/PDGFRA-positive HES occasionally evolves into chronic eosinophilic leukemia or into another form of myeloproliferative neoplasm; however, the development of a malignant lymphoma is very rare. We present a rare case of angioimmunoblastic T-cell lymphoma (AITL) and HES with the FIP1L1/PDGFRA gene rearrangement...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28871382/beyond-stroke-uncommon-causes-of-diffusion-restriction-in-the-basal-ganglia
#9
Alok A Bhatt, Justin L Brucker, Jeevak Almast
In the emergency setting, a regional area of restricted diffusion involving the basal ganglia typically represents an acute infarct due to small vessel occlusion. However, it is important to consider additional differentials, specifically systemic causes. This article will review anatomy of the basal ganglia and pertinent associated vasculature, followed by other entities that can be a cause of restricted diffusion. These include hemolytic uremic syndrome, hypereosinophilic syndrome, fat embolism, meningitis, and hypoxic-ischemic injury...
September 4, 2017: Emergency Radiology
https://www.readbyqxmd.com/read/28757367/hypereosinophilic-syndrome-subtype-predicts-responsiveness-to-glucocorticoids
#10
Paneez Khoury, Annalise O Abiodun, Nicole Holland-Thomas, Michael P Fay, Amy D Klion
BACKGROUND: Glucocorticoids (GCs) are considered first-line treatment for platelet-derived growth factor α (PDGFRA)-negative hypereosinophilic syndromes (HESs). Despite this, little is known about clinical predictors of GC responsiveness in HES. OBJECTIVE: Knowledge of clinical and laboratory predictors of GC response before initiation of GC could lead to more rational selection of subjects with HES for whom earlier institution of second-line and alternative therapies would be appropriate...
July 27, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28701183/ruxolitinib-found-to-cause-eyelash-growth-a-case-report
#11
Julia Song, Alice Song, Trisa Palmares, Michael Song, Harold Song
BACKGROUND: Hypereosinophilic syndrome is a hematologic disorder in which the eosinophils proliferate. Oral Janus kinase inhibitors are known to be effective treating hypereosinophilic syndrome. Janus kinase inhibitors have also demonstrated efficacy in alopecia. Madarosis is a condition in which the eyelashes are missing or absent and can been seen in alopecia patients. CASE PRESENTATION: We present the case of a 77-year-old Asian man who was diagnosed with hypereosinophilic syndrome, refractive to all medications except ruxolitinib...
July 12, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28652675/eosinophilic-myocarditis-and-hypereosinophilic-syndrome
#12
Hanane Boussir, Amine Ghalem, Nabila Ismaili, Noha El Ouafi
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...
July 2017: Journal of the Saudi Heart Association
https://www.readbyqxmd.com/read/28648630/resolution-of-a-steroid-resistant-hypereosinophilic-immune-diathesis-with-mepolizumab-and-concomitant-amelioration-of-a-mixed-thrombotic-microangiopathy
#13
J Justin Mulvey, Cynthia Magro, Amy Chadburn
The anaphylatoxins produced by an unbridled complement cascade in atypical hemolytic uremic syndrome (aHUS) can alter the leukocyte environment in tissues and peripheral blood, causing clinically significant eosinophilia. While the membrane attack complex and C5a anaphlatoxin can be suppressed with anti-C5 biologics, the production of C3a is still capable of driving a destructive hypereosinophilic syndrome in spite of anticomplement therapy. The side-effects of glucocorticoids in treating hypereosinophilic syndrome limit their therapeutic index in long-term treatment and this behooves the use of alternative strategies...
May 2, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28600838/usefulness-of-three-dimensional-echocardiography-in-the-assessment-of-valvular-involvement-in-loeffler-endocarditis
#14
Carlos M Hernandez, Mohammed J Arisha, Amier Ahmad, Ethan Oates, Navin C Nanda, Anil Nanda, Anita Wasan, Beda E Caleti, Cinthia L P Bernal, Sergio M Gallardo
Loeffler endocarditis is a complication of hypereosinophilic syndrome resulting from eosinophilic infiltration of heart tissue. We report a case of Loeffler endocarditis in which three-dimensional transthoracic and transesophageal echocardiography provided additional information to what was found by two-dimensional transthoracic echocardiography alone. Our case illustrates the usefulness of combined two- and three-dimensional echocardiography in the assessment of Loeffler endocarditis. In addition, a summary of the features of hypereosinophilic syndrome and Loeffler endocarditis is provided in tabular form...
July 2017: Echocardiography
https://www.readbyqxmd.com/read/28595207/eosinophilic-myocarditis-presenting-as-acute-coronary-syndrome
#15
Evrim Şimşek, Filiz Özerkan Çakan, Mustafa Akın
Eosinophilic myocarditis (EM) is a rare condition that may result from several heterogeneous eosinophilic diseases, including parasite infection, hypersensitivity reaction, vasculitis, and hypereosinophilic syndrome. Regardless of etiology, the disease may present with various cardiac conditions, such as acute coronary syndrome, heart failure, or arrhythmia. Irreversible endomyocardial fibrosis, which causes restrictive cardiomyopathy, occurs in the late phase of the disease. Early diagnosis and treatment is crucial to prevent disease progression...
June 2017: Türk Kardiyoloji Derneği Arşivi: Türk Kardiyoloji Derneğinin Yayın Organıdır
https://www.readbyqxmd.com/read/28577656/idiopathic-hypereosinophilic-syndrome-with-20-years-of-diagnostic-delay
#16
Miguel Pedro de Queiroz Neto, Fernando Antônio Galvão Gondim Filho
No abstract text is available yet for this article.
April 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28573678/cardiac-involvements-in-hypereosinophilia-associated-syndrome-case-reports-and-a-little-review-of-the-literature
#17
Xuanyi Jin, Chunyan Ma, Shuang Liu, Zhengyu Guan, Yonghuai Wang, Jun Yang
Hypereosinophilia-associated syndrome is a rare group of systemic diseases without certain underlying causes. Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome (CSS), are initial considerations, when underlying causes remains unexplained despite of complete evaluation of hypereosinophilia. In this study, we report two rare cases, one case of HES with Loeffler endocarditis, and the other one of EGPA with restrictive cardiomyopathy mimicking myocardial infarction, to further address differential chief cardiac manifestations between HES and EGPA...
August 2017: Echocardiography
https://www.readbyqxmd.com/read/28546866/a-diagnostic-protocol-designed-for-determining-allergic-causes-in-patients-with-blood-eosinophilia
#18
Jean-François Magnaval, Guy Laurent, Noémie Gaudré, Judith Fillaux, Antoine Berry
BACKGROUND: Blood eosinophilia is a common laboratory abnormality, and its characterization frequently represents a quandary for primary care physicians. Consequently, in France, specialists and particularly hematologists, often must investigate patients who present with blood eosinophilia that often, but not always, occurs because of allergic causes. Both the Departments of Hematology and Parasitology at Toulouse University Hospitals established a collaboration to rule out allergic causes of eosinophilia, particularly helminthiases, prior to initiating more sophisticated investigations...
2017: Military Medical Research
https://www.readbyqxmd.com/read/28546236/therapeutic-effect-of-anti-il-5-on-eosinophilic-myocarditis-with-large-pericardial-effusion
#19
Tengyao Song, David Micheal Jones, Yamen Homsi
Eosinophilic myocarditis (EM) is a rare myocardial disease that results from various eosinophilic diseases, such as idiopathic hypereosinophilic syndrome, helminth infection, medications and vasculitis. Patients with EM may present with different severities, ranging from mild symptoms to a life-threatening condition. Diagnosis of EM is a challenge and requires an extensive workup, including endomyocardial biopsy. Treatment options are limited because EM is rare and there is a lack of randomised controlled trials...
May 24, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28527983/when-a-death-apparently-associated-to-sexual-assault-is-instead-a-natural-death-due-to-idiopathic-hypereosinophilic-syndrome-the-importance-of-gamma-hydroxybutyric-acid-analysis-in-vitreous-humor
#20
Francesco Paolo Busardò, Francesca Portelli, Angelo Montana, Maria Concetta Rotolo, Simona Pichini, Emiliano Maresi
We here report a case involving a 21-year-old female, found dead in a central square of a city in the south of Italy. Initial evidences and circumstances were suggestive of a death associated with a sexual assault. Two peripheral blood and two vitreous humor samples were collected for the purpose of gamma-hydroxybutyric acid (GHB) testing from the dead body at two different post-mortem intervals (PMIs): approximately 2 (t0) and 36 (t1) hours. The obtained results showed that, between t0 and t1, there was an increase of GHB concentrations in peripheral blood and vitreous humor of 66...
May 2017: Legal Medicine
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