Hypereosinophilic disorders.
Ontology highlight
ABSTRACT:
SUBMITTER: Ogbogu PU
PROVIDER: S-EPMC4363101 | biostudies-literature | 2015 Mar-Apr
REPOSITORIES: biostudies-literature
Publications
Hypereosinophilic disorders.
Ogbogu Princess U PU Klion Amy D AD
The journal of allergy and clinical immunology. In practice 20150301 2
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Project description:Eosinophils are leukocytes that are released into the peripheral blood in a phenotypically mature state and are capable of being recruited into tissues in response to appropriate stimuli. Eosinophils, traditionally considered cytotoxic effector cells, are leukocytes recruited into the airways of asthma patients where they are believed to contribute to the development of many features of the disease. This perception, however, has been challenged by recent findings suggesting that eosinophils have also immunomodulatory functions and may be involved in tissue homeostasis and wound healing. Here we describe a transcriptome-based approach-in a limited number of patients and controls-to investigate the activation state of circulating human eosinophils isolated by flow cytometry. We provide an overview of the global expression pattern in eosinophils in various relevant conditions, e.g., eosinophilic asthma, hypereosinophilic dermatological diseases, parasitosis and pulmonary aspergillosis. Compared to healthy subjects, circulating eosinophils isolated from asthma patients differed in their gene expression profile which is marked by downregulation of transcripts involved in antigen presentation, pathogen recognition and mucosal innate immunity, whereas up-regulated genes were involved in response to non-specific stimulation, wounding and maintenance of homeostasis. Eosinophils from other hypereosinophilic disorders displayed a very similar transcriptional profile. Taken together, these observations seem to indicate that eosinophils exhibit non-specific immunomodulatory functions important for tissue repair and homeostasis and suggest new roles for these cells in asthma immunobiology.
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Project description:The eosinophil is a multifunctional granulocyte best known for providing host defense against parasites. Paradoxically, eosinophils are also implicated in the pathogenesis of allergic inflammation, asthma, and hypereosinophilic syndromes. Emerging evidence also supports the potential for harnessing the cytotoxic power of eosinophils and redirecting it to kill solid tumors. Central to eosinophil physiology is interleukin-5 (IL-5) and its receptor (IL-5R) which is composed of a ligand-specific alpha chain (IL-5R?) and the common beta chain (?c). Eosinophil activation can lead to their degranulation, resulting in rapid release of an arsenal of tissue-destructive proinflammatory mediators and cytotoxic proteins that can be both beneficial and detrimental to the host. This review discusses eosinophil immunobiology and therapeutic strategies for targeting of IL-5 and IL-5R, as well as the potential for harnessing eosinophil cytotoxicity as a tumoricide.
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Project description:A 9-year-old boy with hypereosinophilic syndrome (HES) was referred for cardiac magnetic resonance (CMR) imaging following an abnormal echocardiogram that showed a large mass layered on the inferolateral wall of the left ventricle, causing secondary severe mitral regurgitation. Cardiac involvement in HES usually affects the ventricular apex. In our case, CMR confirmed the presence of a large mural thrombus of 0.9 cm × 4.2 cm. This unusual cardiac involvement in HES was diagnosed in its intermediate thrombotic stage. CMR is very sensitive and specific in staging the disease. It explained the etiology of mitral regurgitation and guided therapy, especially when echocardiography was nonconclusive.
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Project description:Hypereosinophilic syndromes (HES) are rare, heterogeneous syndromes characterized by markedly elevated eosinophil counts in the blood and/or tissue and evidence of eosinophil-associated pathology. Although parasitic infections, drug hypersensitivity, and other disorders of defined etiology can present as HES (associated HES), treatment is directed at the underlying cause rather than the eosinophilia itself. A number of additional subtypes of HES have been described, based on clinical and laboratory features. These include (1) myeloid HES-a primary disorder of the myeloid lineage, (2) lymphocytic variant HES-eosinophilia driven by aberrant or clonal lymphocytes secreting eosinophil-promoting cytokines, (3) overlap HES-eosinophilia restricted to a single organ or organ system, (4) familial eosinophilia-a rare inherited form of HES, and (5) idiopathic HES. Since clinical manifestations, response to therapy, and prognosis all differ between HES subtypes, this review will focus on clinical and biological markers that serve as markers of disease activity in HES (excluding associated HES), including those that are likely to be useful only in specific clinical subtypes.
| S-EPMC5743906 | biostudies-literature
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| S-EPMC10087750 | biostudies-literature
Project description:Hypereosinophilic syndrome (HES) is defined by persistently elevated blood eosinophil levels and is associated with evidence of organ damage. Cardiovascular involvement in HES is most commonly associated with Loffler endocarditis (cardiac HES). Cardiac HES is typically characterized by progressive subendocardial fibrosis with overlying mural thrombus formation, leading to restrictive dysfunction of the left ventricle. The thrombus from cardiac HES could result in cardiogenic stroke; however, most of the stroke cases with HES were not associated with huge thromboembolism rather multiple infarcts in the watershed area. The major clinical features of 97 previously reported cases of stroke with HES are as follows: the median age was 52 years, of which 61 (63%) were men; the initial presenting symptoms were neurological (73%), followed by headache (16%), respiratory symptoms (9%), and visual symptoms (9%). Almost half of the cases were diagnosed with cardiac HES. The characteristics of cardiac findings were mural thrombi, endomyocardial fibrosis, and a restrictive pattern of heart failure. Cerebral findings revealed 78 cases (80%) were described as multiple infarctions and 55 cases (57 %) were involved with watershed areas, whereas 11 cases (11％) were described as embolic stroke for one proximal large-vessel occlusion. Regarding treatment, 71 (73%), 28 (29%), and 16 (16%) patients were treated with steroids, anticoagulants, and antiplatelets, respectively. The overall mortality and recovery rates were 11% and 89%, respectively. Physicians should know most cases of stroke with HES are characterized by multiple infarctions in the watershed area, and cardiac HES is not always associated with stroke.
| S-EPMC8636825 | biostudies-literature
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Project description:BackgroundGlucocorticoids (GC) are considered first-line therapy for most patients with hypereosinophilic syndrome (HES). Although response rates are generally high, many patients require moderate to high doses for control of eosinophilia and symptoms, and up to 15% of patients do not respond at all. Despite this, little is known about the mechanisms of GC resistance in patients with HES.ObjectiveTo explore the aetiology of GC resistance in HES.MethodsClinical data and samples from 26 patients with HES enrolled on a prospective study of GC responsiveness and 23 patients with HES enrolled on a natural history study of eosinophilia for whom response to GC was known were analysed retrospectively. Expression of GC receptor isoforms was assessed by quantitative RT-PCR in purified eosinophils. Serum cytokine levels were quantified by suspension array assay in multiplex.ResultsDespite an impaired eosinophil response to GC after 7 days of treatment, the expected rise in absolute neutrophil count was seen in 7/7 GC-resistant patients, suggesting that GC resistance in HES is not a global phenomenon. Eosinophil mRNA expression of glucocorticoid receptor (GR) isoforms (α, β, and P) was similar between GC-sensitive (n = 20) and GC-resistant (n = 9) patients with HES. Whereas geometric mean serum levels were also comparable between GC-r (n = 11) and GC-s (n = 19) for all cytokines tested, serum IL-5 levels were >100 pg/mL only in GC-r patients.Conclusions and clinical relevanceThese data suggest that the mechanism of GC resistance in HES is not due to a global phenomenon affecting all lineages, but may be due, at least in some patients, to impairment of eosinophil apoptosis by increased levels of IL-5.
| S-EPMC6910955 | biostudies-literature