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hypereosinophilic syndrome

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https://www.readbyqxmd.com/read/28094164/disorders-characterized-by-predominant-or-exclusive-dermal-inflammation
#1
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
#2
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
#3
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
#4
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
#5
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 IgG4-RD and 13 L-HES patients. RESULTS: Peripheral blood eosinophilia was present in 8/31 IgG4-RD patients compared to 13/13 L-HES patients (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
#6
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
https://www.readbyqxmd.com/read/27896330/biventricular-thrombus-in-hypereosinophilic-syndrome-presenting-with-shortness-of-breath
#7
Abdul Baqi, Shahan Waheed, Fateh Ali Tipoo, Aamir Hameed Khan
A 48 years old male presented to clinic with 12 months of low grade fever with shortness of breath which has progressively worsened with no associated weight loss, night sweats or loss of appetite. There was no prior history of chronic illness before the current illness. Laboratory workup revealed a high white blood cell count with predominant eosinophils. Chest X-ray was normal. Transthoracic echocardiography and Cardiac Magnetic Resonance showed biventricular thrombi. On further extensive workup the findings were consistent with hypereosinophilic syndrome...
June 2016: Turkish Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27887955/treatment-of-hypereosinophilic-syndrome-with-cutaneous-involvement-with-the-jak-inhibitors-tofacitinib-and-ruxolitinib
#8
Brett King, Alfred Ian Lee, Jaehyuk Choi
No abstract text is available yet for this article.
November 22, 2016: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/27883350/loeffler-endocarditis-in-young-woman-a-case-report
#9
Natalia Y Osovska, Natalia V Kuzminova, Irina I Knyazkova
Loeffler endocarditis is a rare acquired endocardial and myocardial disease characterized by a sharp decrease in the compliance of either or both ventricles with an acute diastolic dysfunction and massive mural thrombosis. This disease is presented in the classification of cardiomyopathies and is a variant of restrictive cardiomyopathy. Today Loeffler endocarditis is considered as a manifestation of hypereosinophilic syndrome with predominant heart involvement. The life-time diagnosis of myocardial injury due to eosinophilic infiltration is rare, or it is diagnosed at the stage of necrotizing endomyocarditis, when the treatment is no longer effective...
November 25, 2016: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/27866580/eosinophilia
#10
REVIEW
Anna Kovalszki, Peter F Weller
Eosinophilia is defined as elevation of eosinophils in the bloodstream (450-550 cell/μL). There are many reasons for eosinophilia to exist, including parasitic disease, allergic disease, autoimmune, connective tissue disease, rheumatologic disease, primary eosinophilia such as hypereosinophilic syndrome, and as part of a malignant state. Primary care physicians should have an understanding of the variety of diseases or situations that can produce eosinophilia and know in what setting referral to specialty care may be warranted...
December 2016: Primary Care
https://www.readbyqxmd.com/read/27846662/-the-lymphoid-variant-of-hes-l-hes-as-differential-diagnose-of-severe-asthma-in-childhood
#11
T Leu, S Rauthe, C Wirth, H-U Simon, V Kunzmann, H Hebestreit, S Kunzmann
Based on a case report an overview on the differential diagnostic considerations with respect to blood hypereosinophilia (HE) and hypereosinophilic syndromes (HES) in childhood is given. A 13-year-old boy was admitted for the clarification of an asthma. In the blood count an increased HE with 3 500/µl (30%) was found along with elevated total serum IgE and IL-5 level (2 000 IU/ml and 17 pg/ml). Lung function showed an obstruction (FEV1 38%). Radiologically the picture of bronchiectasis and mucus pluggine appeared...
November 2016: Klinische Pädiatrie
https://www.readbyqxmd.com/read/27825424/radiological-and-clinical-features-of-eosinophilic-granulomatosis-with-polyangiitis
#12
Xiao-Li Xu, Wei Song, Xin Sui, Lan Song, Qian-Ni DU, Xiao Wang
Eosinophilic granulomatosis with polyangiitis(EGPA),also known as Churg-Strauss syndrome,is a clinically rare small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs),characterized by asthma,disseminated necrotizing vasculitis,extravascular granulomas,peripheral eosinophilia,and tissue eosinophilia. This article reviews the pathology,imaging,and clinical features of EGPA.
October 10, 2016: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/27803915/idiopathic-hypereosinophilic-syndrome-presenting-with-severe-vasculitis-successfully-treated-with-imatinib
#13
Paolo Fraticelli, Alain Kafyeke, Massimo Mattioli, Giuseppe Pio Martino, Marta Murri, Armando Gabrielli
Idiopathic hypereosinophilic syndrome (HES) is a rare disorder characterized by peripheral eosinophilia exceeding 1500/mm(3), a chronic course, absence of secondary causes, and signs and symptoms of eosinophil-mediated tissue injury. One of the best-characterized forms of HES is the one associated with FIP1L1-PDGFRA gene rearrangement, which was recently demonstrated as responsive to treatment with the small molecule kinase inhibitor drug, imatinib mesylate. Here, we describe the case of a 51-year-old male, whose symptoms satisfied the clinical criteria for HES with cutaneous and cardiac involvement and who also presented with vasculitic brain lesions and retroperitoneal bleeding...
October 16, 2016: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/27801321/-clinical-characteristics-and-long-term-therapeutic-effects-of-60-patients-with-idiopathic-hypereosinophilic-syndrome-in-a-single-center
#14
S Q Qu, T J Qin, Z F Xu, Y Zhang, X F Ai, B Li, H L Zhang, L W Fang, L J Pan, N B Hu, Z J Xiao
Objective: To analyze the long term outcome of patients with hypereosinophilic syndrome(HES). Methods: The clinical characteristics and efficacy of 60 newly diagnosed HES patients who received corticosteroids(CS)monotherapy were retrospectively analyzed. The survival and death causes of patients were obtained by follow- up. Results: Of all 60 HES patients, 45 were male and 15 female. The median age was 38(11-80)years old. The most frequent organ involvement of HES occurred in cutaneous(55.0%), gastrointestinal(40...
October 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/27759698/occlusive-nonvasculitic-vasculopathy-a-review
#15
Mar Llamas-Velasco, Victoria Alegría, Ángel Santos-Briz, Lorenzo Cerroni, Heinz Kutzner, Luis Requena
We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Different disorders may produce nonvasculitic occlusive vasculopathy in cutaneous blood and lymphatic vessels, including embolization due to cholesterol and oxalate emboli, cutaneous intravascular metastasis from visceral malignancies, atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis, intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material...
October 18, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27740624/rhoh-is-a-negative-regulator-of-eosinophilopoiesis
#16
Christina Stoeckle, Barbara Geering, Shida Yousefi, Saša Rožman, Nicola Andina, Charaf Benarafa, Hans-Uwe Simon
Eosinophils are frequently elevated in pathological conditions and can cause tissue damage and disease exacerbation. The number of eosinophils in the blood is largely regulated by factors controlling their production in the bone marrow. While several exogenous factors, such as interleukin-5, have been described to promote eosinophil differentiation, comparatively little is known about eosinophil-intrinsic factors that control their de novo generation. Here, we report that the small atypical GTPase RhoH is induced during human eosinophil differentiation, highly expressed in mature blood eosinophils and further upregulated in patients suffering from a hypereosinophilic syndrome...
December 2016: Cell Death and Differentiation
https://www.readbyqxmd.com/read/27733964/gastrointestinal-and-hepatic-involvement-in-hypereosinophilic-syndrome
#17
Faisal Inayat, Abu Hurairah
The objective of this investigation was to study the gastrointestinal and hepatic involvement in hypereosinophilic syndrome (HES). Gastrointestinal or hepatic involvement is estimated to affect up to one-third of patients with HES, although most of the clinical evidence has been derived from case reports. In literature, HES presenting with hepatitis and jaundice with subsequent development of colitis is a rare clinicopathologic entity. Given the clinical implications, physicians should include HES among differentials in these types of presentations...
August 31, 2016: Curēus
https://www.readbyqxmd.com/read/27728750/lymphocytic-variant-of-hypereosinophilic-syndrome-with-deep-vein-thrombosis
#18
Vivek Thomas
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27728301/idiopathic-hypereosinophilic-syndrome
#19
Anil Mangal, C L Nawal, M K Agarwal, Pradeep Mital
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27728295/a-case-of-hypereosinophillic-syndrome-parading-as-copd
#20
S Banerjee, S Ganguly
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
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