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hypereosinophilic syndrome

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https://www.readbyqxmd.com/read/28401108/a-case-of-lymphocytic-variant-hypereosinophilic-syndrome-with-sub-diagnostic-systemic-mastocytosis
#1
Preetesh Jain, Sa A Wang, C Cameron Yin, Yasmin Abaza, Srdan Verstovsek, Zeev Estrov
No abstract text is available yet for this article.
March 2017: Blood Research
https://www.readbyqxmd.com/read/28349221/total-artificial-heart-implantation-for-biventricular-failure-due-to-eosinophilic-myocarditis
#2
Masashi Kawabori, Chitaru Kurihara, Yair Miller, Kent A Heck, Roberta C Bogaev, Andrew B Civitello, William E Cohn, O H Frazier, Jeffrey A Morgan
Idiopathic hypereosinophilic syndrome is a condition of unknown etiology characterized by proliferation of eosinophils and their infiltration into tissues. Although cardiac involvement is rare, eosinophilic myocarditis can lead to life-threating fulminant congestive heart failure. Treatment of patients with eosinophilic myocarditis is challenging as heart failure can be caused by biventricular dysfunction. To our knowledge, this is the first case reported in the literature describing a patient with acute severe biventricular heart failure caused by eosinophilic myocarditis with mural left ventricular apical thrombus who was successfully treated with implantation of a total artificial heart as a bridge to heart transplant...
March 27, 2017: Journal of Artificial Organs: the Official Journal of the Japanese Society for Artificial Organs
https://www.readbyqxmd.com/read/28347583/hypereosinophilic-syndrome-as-a-rare-cause-of-reversible-biventricular-heart-failure
#3
Maria Bonou, Chris J Kapelios, George Benetos, Ioannis Moyssakis, Nefeli Giannakopoulou, Panagiotis Diamantopoulos, Penelope Korkolopoulou, Eleni Variami, John Barbetseas
Hypereosinophilic syndrome is a rare entity that can develop secondary to overproduction of eosinophilopoietic cytokines or as idiopathic disease. Cardiac involvement, which occurs often, is divided into 3 stages, the latter of which is nonreversible and leads to severe heart failure. Early detection and treatment of the syndrome is essential. For this reason, genetic testing for the FIP1L1-PDGFRA fusion gene has recently been added to the diagnostic algorithm. Patients with this mutation are at increased risk for the development of cardiac involvement and typically respond to treatment with the tyrosine kinase inhibitor imatinib mesylate...
January 20, 2017: Canadian Journal of Cardiology
https://www.readbyqxmd.com/read/28337408/eosinophilic-granulomatosis-with-polyangiitis-formerly-known-as-churg-strauss-syndrome-as-a-differential-diagnosis-of-hypereosinophilic-syndromes
#4
Yuri Albuquerque Pessoa Santos, Bruno Rangel Antunes Silva, Pollyanna Natividade Zanconato Barros Assis Lira, Luiz Carlos Aguiar Vaz, Thiago Thomaz Mafort, Leonardo Palermo Bruno, Agnaldo José Lopes
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic disease situated between primary small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Here, we present a case of EGPA in a 38-year-old male, with a previous diagnosis of asthma, who presented with fever, migratory lung infiltrates and systemic eosinophilia that was refractory to previous courses of antibiotics. This case highlights the importance of the primary care physician understanding the differential diagnosis of pulmonary eosinophilic syndromes...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28321782/targeting-interleukin-5-or-interleukin-5r%C3%AE-safety-considerations
#5
REVIEW
Diego Bagnasco, Matteo Ferrando, Marco Caminati, Alice Bragantini, Francesca Puggioni, Gilda Varricchi, Giovanni Passalacqua, Giorgio Walter Canonica
Asthma is a highly prevalent chronic disease of the airways; approximately 10% of patients with asthma will experience a severe form of the disease. New understanding of the pathogenesis of asthma has enabled the development of novel drugs and provided hope for patients with asthma. Interleukin (IL)-5 and IL-5 receptor subunit α (IL-5-Rα) plays a crucial role in the development, maturation, and operation of eosinophils so were the first important therapeutic target of these new drugs. While the results of early clinical trials of these drugs were not promising, results improved once researchers discovered the drugs worked best in patients with high eosinophil levels...
March 20, 2017: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
https://www.readbyqxmd.com/read/28290180/non-bacterial-thrombotic-endocarditis-of-aortic-valve-due-to-hypereosinophilic-syndrome
#6
Harveen Lamba, Salil Deo, Salah Altarabsheh, Yakov Elgudin, Allen Markowitz, Soon Park
Hypereosinophilic syndrome (HES) is a rare hematological disorder, which may present with cardiac involvement. The case is presented of a 61-year-old male patient with isolated aortic stenosis secondary to non-bacterial thrombotic endocarditis and HES. The patient underwent successful aortic valve replacement with a bioprosthesis and remained recurrence-free at the 18-month follow up. A review of the current literature is also presented and cardiac manifestations, clinical presentation and surgical issues in the care of patients with this rare condition are discussed...
November 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28290178/multiple-valvular-complications-of-hypereosinophilic-syndrome
#7
Zoltán Pozsonyi, Szabolcs Benedek, Pál Sármán, Lívia Jánoskuti, Tivadar Hüttl, Astrid Apor
Endomyocardial fibrosis (EMF) is the most common cardiac abnormality in hyeperosinophilic syndrome (HES), sometimes complicated with mitral valve disease. Mitral valve disease without ventricular manifestation is very rare, however. Case reports link HES to prosthetic valve thrombosis (PVT), but the optimal type of prosthetic valve in HES is not known. Herein is reported the case of a young female HES patient with secondary mitral valve degeneration and severe regurgitation. A mechanical prosthetic valve was implanted six months after she was diagnosed with HES, but despite anticoagulation and antiplatelet therapy she developed PVT three months later...
November 2016: Journal of Heart Valve Disease
https://www.readbyqxmd.com/read/28284183/multiparametric-cardiac-magnetic-resonance-imaging-cmr-for-the-diagnosis-of-loeffler-s-endocarditis-a-case-report
#8
Mareike Gastl, Patrick Behm, Christoph Jacoby, Malte Kelm, Florian Bönner
BACKGROUND: Endocarditis parietalis fibroplastica Löfflein (EPF) is a rare form of primary restrictive cardiomyopathy with poor prognosis. It is generally caused by hypereosinophilic syndrome with eosinophilic penetration of the heart. This leads to congestive heart failure in three different stages. As a frequent manifestation of neoplastic diseases, cardiac involvement means poor prognosis. CASE PRESENTATION: The present report deals with a case of EPF caused by non-specified T-cell lymphoma (T-NOS)...
March 11, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28263223/hypereosinophilic-syndrome-after-liver-transplantation-a-case-report-and-a-review-of-the-literature
#9
Wiebke Aulbert, Robin Kobbe, Christian Breuer, Andrea Briem-Richter, Hansjörg Schäfer, Florian Brinkert, Anne Dettmar, Markus J Kemper, Enke Grabhorn
Persistently elevated eosinophil granulocytes in the peripheral blood in children is challenging due to a complex diagnosis especially after solid organ transplantation and can lead to difficulties in finding an underlying causative factor.We report a 12-year-old boy who developed severe hypereosinophilia eleven years after liver transplantation (LT) due to biliary atresia. Accompanying symptoms were recurrent fever, fatigue, elevated liver enzymes, abdominal pain and significant weight loss. After exclusion of secondary causes of eosinophilia, an idiopathic hypereosinophilic syndrome (I-HES) was diagnosed...
March 4, 2017: Transplantation
https://www.readbyqxmd.com/read/28219563/repeated-prosthetic-mitral-valve-thrombosis-in-fluke%C3%A2-infection-induced-hypereosinophilic-syndrome
#10
Yiran Zhang, Liang Ma
A 43-year-old female patient with mitral valve stenosis and hypereosinophilia on admission received mechanical mitral valve replacement. During the 2 years after the first surgery, the patient underwent two mitral valve revision operations for repeated prosthetic mitral valve thrombosis. During the 2-year period, the patient's absolute eosinophil count was found elevated repeatedly. Finally, fluke antibody detection revealed fluke infection and, after praziquantel treatment, a normal level of absolute eosinophil count has been maintained and recurrent thrombosis has not occurred for 18 months...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28178599/the-targeted-eosinophil-lowering-effects-of-dexpramipexole-in-clinical-studies
#11
Steven I Dworetzky, Gregory T Hebrank, Donald G Archibald, Ian J Reynolds, Wildon Farwell, Michael E Bozik
Dexpramipexole, an orally bioavailable small molecule previously under clinical development in amyotrophic lateral sclerosis, was observed during routine safety hematology monitoring to demonstrate pronounced, dose- and time-dependent eosinophil-lowering effects, with minor reductions on other leukocyte counts. Analysis of hematology lab values across two double-blind, randomized placebo-controlled clinical trials at total daily doses ranging from 50mg to 300mg demonstrated that dexpramipexole consistently and markedly lowered peripheral blood eosinophils...
January 16, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28174010/a-case-of-t-cell-lymphoproliferative-disorder-associated-with-hypereosinophilia-with-excellent-response-to-mycophenolate-mofetil
#12
Amr Hanbali, Marwan Shaheen, Feras Alfraih, Wafa Al-Otaibi, Riad El Fakih, Tarek Owaidah, Syed Ahmed
Hypereosinophilic syndrome (HES) is a group of rare blood disorders characterized by a persistent elevation of blood eosinophil count ⩾1.5×10(9)/L and clinical manifestations attributable to eosinophilia or tissue hypereosinophilia. Lymphocytic variant of HES (HES-L) is a known subtype according to World Health Organization classification. It is well documented in the literature that patients with HES-L are predisposed to develop T-cell lymphoma. We report a case of T-cell lymphoproliferation associated with hypereosinophilia, which has been successfully treated with mycophenolate mofetil, with resolution of skin lesions and normalization of eosinophil count and immunoglobulin E level...
February 1, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28168065/rapid-change-in-mental-status-in-a-patient-with-hypereosinophilia
#13
Hanyin Wang, John K Erban
We present the case of a 48-year-old female with acute onset altered mental status, who was found to have eosinophilia, elevated troponin, and embolic strokes. Extensive testing for autoimmune, infectious, and coronary artery etiologies was unremarkable. After a cardiac MRI revealed focal myocardial hyperenhancement, the patient underwent an endomyocardial biopsy with findings consistent with eosinophilic myocarditis. The patient was diagnosed of idiopathic hypereosinophilic syndrome and started on prednisone and apixaban...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28111307/jak1-gain-of-function-causes-an-autosomal-dominant-immune-dysregulatory-and-hypereosinophilic-syndrome
#14
Kate L Del Bel, Robert J Ragotte, Aabida Saferali, Susan Lee, Suzanne M Vercauteren, Sara A Mostafavi, Richard A Schreiber, Julie S Prendiville, Min S Phang, Jessica Halparin, Nicholas Au, John M Dean, John J Priatel, Emily Jewels, Anne K Junker, Paul C Rogers, Michael Seear, Margaret L McKinnon, Stuart E Turvey
No abstract text is available yet for this article.
January 19, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28094164/disorders-characterized-by-predominant-or-exclusive-dermal-inflammation
#15
Mark R Wick
Some cutaneous inflammatory disorders are typified by a predominant or exclusive localization in the dermis. They can be further subdivided by the principal cell types into lymphocytic, neutrophilic, and eosinophilic infiltrates, and mixtures of them are also seen in a proportion of cases. This review considers such conditions. Included among the lymphoid lesions are viral exanthems, pigmented purpuras, gyrate erythemas, polymorphous light eruption, lupus tumidus, and cutaneous lymphoid hyperplasia. Neutrophilic infiltrates are represented by infections, Sweet syndrome, pyoderma gangrenosum, and hidradenitis suppurativa, as well as a group of so-called "autoinflammatory" dermatitides comprising polymorphonuclear leukocytes...
December 14, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28070732/the-clinical-and-laboratory-spectrum-of-dedicator-of-cytokinesis-8-immunodeficiency-syndrome-in-patients-with-a-unique-mutation
#16
Arnon Broides, Amarilla B Mandola, Jacov Levy, Baruch Yerushalmi, Vered Pinsk, Michal Eldan, George Shubinsky, Nurit Hadad, Rachel Levy, Amit Nahum, Miriam Ben-Harosh, Atar Lev, Amos Simon, Raz Somech
Mutations in the dedicator of cytokinesis 8 (DOCK8) gene cause a combined immunodeficiency usually diagnosed as autosomal recessive hyper IgE syndrome. We sought to reveal the varying manifestations in patients with a unique mutation in DOCK8 gene by a retrospective medical record review. Ten patients from five consanguineous families and three tribes were included. Seven patients were homozygous for the c.C5134A, p.S1711X mutation, and the remaining three patients were their siblings manifesting hyper IgE syndrome features without a genetic diagnosis...
January 10, 2017: Immunologic Research
https://www.readbyqxmd.com/read/28065613/anca-negative-churg-strauss-syndrome-presenting-as-acute-multiple-cerebral-infarcts-a-case-report
#17
Klearchos Psychogios, Ilias Evmorfiadis, Spyros Dragomanovits, Athanasios Stavridis, Konstantinos Takis, Loukas Kaklamanis, Stathis Pantelis
Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28052805/hypereosinophilic-syndrome
#18
REVIEW
Heung R Noh, Gil G Magpantay
BACKGROUND: Hypereosinophilic syndrome (HES) is a myeloproliferative disorder characterized by persistent eosinophilia associated with multiple organ damage. OBJECTIVE: To increase awareness of the rare but potentially life-threatening disease and to provide a brief overview of the clinical presentation, diagnosis, and management of HES. METHODS: Pertinent data from the patient's clinical course was incorporated with salient articles on the topic...
January 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28005278/igg4-related-disease-and-lymphocyte-variant-hypereosinophilic-syndrome-a-comparative-case-series
#19
Mollie N Carruthers, Sujin Park, Graham W Slack, Bakul I Dalal, Brian F Skinnider, David F Schaeffer, Jan P Dutz, Joanna K Law, Fergal Donnellan, Vladimir Marquez, Michael Seidman, Patrick C Wong, Andre Mattman, Luke Y C Chen
OBJECTIVE: To compare the clinical and laboratory features of IgG4-related disease (IgG4-RD) and lymphocyte-variant hypereosinophilic syndrome (L-HES), two rare diseases that often present with lymphadenopathy, gastrointestinal symptoms, eosinophilia, and elevated immunoglobulins/IgE. METHOD: Comparative case series of 31 patients with IgG4-RD and 13 patients with L-HES. RESULTS: Peripheral blood eosinophilia was present in eight of 31 patients with IgG4-RD compared to 13 of 13 patients with L-HES (median eosinophils 0...
December 22, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27911676/tolerance-and-efficacy-with-simultaneous-use-of-two-monoclonal-antibodies-for-a-patient-with-hypereosinophilic-syndrome-and-ulcerative-colitis
#20
Bhavisha Patel, Joseph H Butterfield, Catherine R Weiler, Sunanda V Kane
The development and utilization of monoclonal antibodies (mAbs) have been of great interest in all fields of medicine. A substantial increase in the production and development of mAbs has occurred because these biologic agents are proving to be effective and less toxic given their targeted mechanism of action. However, data are limited on coadministration of two or more mAbs. With the increasing availability of mAbs and the comorbidities of some patients, assessment is needed of the ability to safely use multiple mAbs for an individual patient...
December 2016: Monoclonal Antibodies in Immunodiagnosis and Immunotherapy
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