Medicine › Rheumatology

Eosinophilic Disorders and Syndromes

Works Count: 34971

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Description

This cluster of papers focuses on the hypereosinophilic syndrome and related disorders, including the molecular pathogenesis, classification, and therapeutic management. It covers topics such as fusion genes (e.g., FIP1L1-PDGFRA), imatinib mesylate treatment, cardiac manifestations, and the classification of eosinophilic disorders.

Keywords

Hypereosinophilic Syndrome; Fusion Genes; Imatinib Mesylate; Eosinophilia; PDGFRA; Classification; Molecular Pathogenesis; Cardiac Manifestations; Therapeutic Management; Eosinophilic Disorders

The idiopathic hypereosinophilic syndrome

1994-05-15

PF Weller , Glenn J. Bubley

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RANTES is a chemotactic and activating factor for human eosinophils.

1993-04-15

Rafeul Alam , Susan Stafford , Paul Forsythe +4 more

Abstract RANTES is a member of the 8-kDa cytokine family that has been shown to possess chemotactic activity for monocytes and CD4 T cells. In this study, we investigated whether … Abstract RANTES is a member of the 8-kDa cytokine family that has been shown to possess chemotactic activity for monocytes and CD4 T cells. In this study, we investigated whether RANTES could affect eosinophil chemotaxis and function. Peripheral blood eosinophils from blood donors were isolated on Percoll gradients to &gt; 98% purity and then used for chemotaxis, flow cytometry, eosinophil cationic protein release assay, and survival assay. We found that RANTES is chemotactic for eosinophils at 10(-9) to 10(-8) M concentrations. RANTES elicited 65% of the chemotactic response to 10(-7) M platelet-activating factor in all experiments. The mechanism of chemotaxis was investigated by studying the expression of adhesion molecules on eosinophils by flow cytometry. We found that RANTES up-regulated the expression of CD11b/CD18 on eosinophils in a dose-dependent manner. In another set of experiments, purified eosinophils incubated with various concentrations of RANTES released eosinophil cationic protein as measured by a RIA. We also investigated the effect of RANTES on eosinophil density. Leukocytes were incubated in the presence or absence of RANTES, and the distribution of eosinophils on discontinuous Percoll gradients was then examined. We found that eosinophils became hypodense (&lt; 1.085) when incubated in RANTES. However, unlike IL-3, RANTES did not affect the survival of eosinophils in a 4-day culture system. Thus, we established that RANTES is a chemotactic and activating factor for eosinophils.

Convenient assay for interferons

1981-02-01

Sara Rubinstein , P C Familletti , Sidney Pestka

A convenient assay for interferons based on reduction of cytopathic effect was developed. The number of manipulations and the lengths of the various incubation steps were reduced to a minimum. … A convenient assay for interferons based on reduction of cytopathic effect was developed. The number of manipulations and the lengths of the various incubation steps were reduced to a minimum. The assay is simple to perform and can be completed within 16 h. Moreover, it can be used with various types of cells and a variety of viruses.

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Recent Developments in Myofibroblast Biology

2012-03-03

Boris Hinz , Sem H. Phan , Victor J. Thannickal +6 more

The discovery of the myofibroblast has opened new perspectives for the comprehension of the biological mechanisms involved in wound healing and fibrotic diseases. In recent years, many advances have been … The discovery of the myofibroblast has opened new perspectives for the comprehension of the biological mechanisms involved in wound healing and fibrotic diseases. In recent years, many advances have been made in understanding important aspects of myofibroblast basic biological characteristics. This review summarizes such advances in several fields, such as the following: i) force production by the myofibroblast and mechanisms of connective tissue remodeling; ii) factors controlling the expression of α-smooth muscle actin, the most used marker of myofibroblastic phenotype and, more important, involved in force generation by the myofibroblast; and iii) factors affecting genesis of the myofibroblast and its differentiation from precursor cells, in particular epigenetic factors, such as DNA methylation, microRNAs, and histone modification. We also review the origin and the specific features of the myofibroblast in diverse fibrotic lesions, such as systemic sclerosis; kidney, liver, and lung fibrosis; and the stromal reaction to certain epithelial tumors. Finally, we summarize the emerging strategies for influencing myofibroblast behavior in vitro and in vivo, with the ultimate goal of an effective therapeutic approach for myofibroblast-dependent diseases.

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THROMBOSIS IN SYSTEMIC LUPUS ERYTHEMATOSUS DESPITE CIRCULATING ANTICOAGULANTS.

1963-09-01

Bowie Ej , Thompson Jh , Pascuzzi Ca +1 more

The Hypereosinophilic Syndromes

1968-06-01

W. Rod Hardy

This report concerns three individuals with a similar illness characterized by the following: a striking leukocytosis due primarily to increased numbers of mature eosinophils; cardiac or pulmonary symptoms, or both; … This report concerns three individuals with a similar illness characterized by the following: a striking leukocytosis due primarily to increased numbers of mature eosinophils; cardiac or pulmonary symptoms, or both; and hepatosplenomegaly. Two of the patients died; the third remains in clinical good health. Distinctive morphologic abnormalities were found in the heart, bone marrow, liver, and spleen. Similar cases have often been reported as examples of eosinophilic leukemia. However, review of the pertinent evidence is not thought to support this concept; rather, it suggests an association with Löffler's syndrome and Löffler's endocarditis parietalis fibroplastica. To emphasize that such patients are part of a continuum, we propose the term "hypereosinophilic syndromes" to encompass the entire group.

The Idiopathic Hypereosinophilic Syndrome

1982-07-01

Anthony S. Fauci

The idiopathic hypereosinophilic syndrome (HES) represents a heterogeneous group of disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. Fifty patients with the … The idiopathic hypereosinophilic syndrome (HES) represents a heterogeneous group of disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. Fifty patients with the idiopathic HES were studied over 11 years at the National Institutes of Health. Multiple organ systems were involved; bone marrow hypereosinophilia was common to all patients, but the most severe clinicopathologic involvement was of the heart and nervous system. Postmortem gross pathologic examination of the hearts of patients with idiopathic and nonidiopathic HES suggested that the common mechanism of cardiac disease is the eosinophilia. Endomyocardial biopsy findings showed that the endothelial cells in the endocardium and of the microvasculature were the primary targets of the tissue damage. This damage initiates thrombosis; endocardial fibrosis and restrictive endomyocardopathy may follow. Invitro culture of circulating eosinophil colony-forming units showed some normal studies, some studies showing increased progenitor cells committed to eosinophil development,and others showing an excess production of eosinophil colony-stimulating factor. Chemotherapy to lower the eosinophil counts has resulted in marked improvement of HES prognosis, as have agressive medical and surgical approaches to cardiovascular complications.

Hypereosinophilic syndrome: A multicenter, retrospective analysis of clinical characteristics and response to therapy

2009-11-13

Princess U. Ogbogu , Bruce S. Bochner , Joseph H. Butterfield +7 more

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An Investigation of the Cause of the Eosinophilia–Myalgia Syndrome Associated with Tryptophan Use

1990-08-09

Edward A. Belongia , Craig W. Hedberg , Gerald J. Gleich +7 more

The eosinophilia—myalgia syndrome is a newly recognized illness that has been associated with the consumption of tryptophan products. It is not known whether the cause is related to the tryptophan … The eosinophilia—myalgia syndrome is a newly recognized illness that has been associated with the consumption of tryptophan products. It is not known whether the cause is related to the tryptophan itself or to chemical constituents introduced by the manufacturing process.

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High-affinity IgE receptor on eosinophils is involved in defence against parasites

1994-01-13

Abdelilah S. Gounni , Bouchaïb Lamkhioued , Kenichi Ochiai +5 more

Interleukin-1–Receptor Antagonist in the Muckle–Wells Syndrome

2003-06-18

Philip N. Hawkins , Helen J. Lachmann , Michael McDermott

To the Editor: Studies of hereditary inflammatory disorders have identified novel genes and pathways that may be involved in inflammation and apoptosis generally. Mutations in one such gene, variou... To the Editor: Studies of hereditary inflammatory disorders have identified novel genes and pathways that may be involved in inflammation and apoptosis generally. Mutations in one such gene, variou...

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The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature.

1975-01-01

Michael J. Chusid , David C. Dale , Burton C. West +1 more

Several closely related disease entities make up the idiopathic hypereosinophilic syndrome (HES). The syndrome is manifest by persistent and prolonged eosinophilia with organ damage. A group of 14 patients had … Several closely related disease entities make up the idiopathic hypereosinophilic syndrome (HES). The syndrome is manifest by persistent and prolonged eosinophilia with organ damage. A group of 14 patients had hematologic, cardiac, and neurologic abnormalities attributable to this disease. Patient survival and response to chemotherapy was significantly better in this group than in previously reported patients. The etiology of HES remains unknown, as does the mechanism of tissue damage.

Targeting eosinophils in allergy, inflammation and beyond

2013-01-21

Patricia C. Fulkerson , Marc E. Rothenberg

cDNA sequence and chromosomal localization of human platelet-derived growth factor A-chain and its expression in tumour cell lines

1986-04-01

Christer Betsholtz , Ann Johnsson , Carl‐Henrik Heldin +7 more

A Tyrosine Kinase Created by Fusion of the<i>PDGFRA</i>and<i>FIP1L1</i>Genes as a Therapeutic Target of Imatinib in Idiopathic Hypereosinophilic Syndrome

2003-03-26

Jan Cools , Daniel J. DeAngelo , Jason Gotlib +7 more

Idiopathic hypereosinophilic syndrome involves a prolonged state of eosinophilia associated with organ dysfunction. It is of unknown cause. Recent reports of responses to imatinib in patients with the syndrome suggested … Idiopathic hypereosinophilic syndrome involves a prolonged state of eosinophilia associated with organ dysfunction. It is of unknown cause. Recent reports of responses to imatinib in patients with the syndrome suggested that an activated kinase such as ABL, platelet-derived growth factor receptor (PDGFR), or KIT, all of which are inhibited by imatinib, might be the cause. We treated 11 patients with the hypereosinophilic syndrome with imatinib and identified the molecular basis for the response. Nine of the 11 patients treated with imatinib had responses lasting more than three months in which the eosinophil count returned to normal. One such patient had a complex chromosomal abnormality, leading to the identification of a fusion of the Fip1-like 1 (FIP1L1) gene to the PDGFRalpha (PDGFRA) gene generated by an interstitial deletion on chromosome 4q12. FIP1L1-PDGFRalpha is a constitutively activated tyrosine kinase that transforms hematopoietic cells and is inhibited by imatinib (50 percent inhibitory concentration, 3.2 nM). The FIP1L1-PDGFRA fusion gene was subsequently detected in 9 of 16 patients with the syndrome and in 5 of the 9 patients with responses to imatinib that lasted more than three months. Relapse in one patient correlated with the appearance of a T674I mutation in PDGFRA that confers resistance to imatinib. The hypereosinophilic syndrome may result from a novel fusion tyrosine kinase - FIP1L1-PDGFRalpha - that is a consequence of an interstitial chromosomal deletion. The acquisition of a T674I resistance mutation at the time of relapse demonstrates that FIP1L1-PDGFRalpha is the target of imatinib. Our data indicate that the deletion of genetic material may result in gain-of-function fusion proteins.

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MALIGNANT HISTIOCYTOSIS OF THE INTESTINE: A T-CELL LYMPHOMA

1985-09-01

P G Isaacson , Jo Spencer , C. E. Connolly +7 more

Drug-induced pseudolymphoma and drug hypersensitivity syndrome (drug rash with eosinophilia and systemic symptoms: DRESS)

1996-12-01

Hélène Bocquet , M. Bagot , Jean Claude Roujeau

Since the first description by Saltzstein in 1959, the denomination of drug-induced pseudolymphoma was used to describe two cutaneous adverse drug reactions with a histological picture mimicking malignant lymphoma. On … Since the first description by Saltzstein in 1959, the denomination of drug-induced pseudolymphoma was used to describe two cutaneous adverse drug reactions with a histological picture mimicking malignant lymphoma. On the basis of clinical presentation, this term includes two different patterns: (1) hypersensitivity syndrome which begins acutely in the first 2 months after the initiation of the drug and associates fever, a severe skin disease with characteristic infiltrated papules and facial edema or an exfoliative dermatitis, lymphadenopathy, hematologic abnormalities (hypereosinophilia, atypical lymphocytes) and organ involvement such as hepatitis, carditis, interstitial nephritis, or interstitial pneumonitis. The cutaneous histological pattern shows a lymphocytic infiltrate, sometimes mimicking a cutaneous lymphoma, and the mortality rate is about 10%. When organ involvement exists, corticosteroids are often prescribed with dramatic improvement. Relapses may occur. (2) drug-induced pseudolymphoma which has a more insidious beginning with nodules and infiltrated plaques appearing several weeks after the beginning of the drug without constitutional symptoms. A pseudolymphoma pattern is seen on cutaneous histological slides. Complete improvement is usual after drug withdrawal, but a delayed lymphoma is possible. To decrease the ambiguity of the denomination of hypersensitivity syndrome, we propose the term of DRESS (Drug Rash with Eosinophilia and Systemic Symptoms).

Eosinophils: changing perspectives in health and disease

2012-11-16

Helene F. Rosenberg , Kimberly D. Dyer , Paul S. Foster

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Cloning of the human eosinophil chemoattractant, eotaxin. Expression, receptor binding, and functional properties suggest a mechanism for the selective recruitment of eosinophils.

1996-02-01

Paul Ponath , Suofu Qin , D J Ringler +7 more

The CC chemokine eotaxin, identified in guinea pigs and also recently in mice, may be a key element for the selective recruitment of eosinophils to certain inflamed tissues. Using a … The CC chemokine eotaxin, identified in guinea pigs and also recently in mice, may be a key element for the selective recruitment of eosinophils to certain inflamed tissues. Using a partial mouse eotaxin CDNA probe, the human eotaxin gene was cloned and found to be 61.8 and 63.2% identical at the amino acid level to guinea pig and mouse eotaxin. Human eotaxin protein was a strong and specific eosinophil chemoattractant in vitro and was an effective eosinophil chemoattractant when injected into the skin of a rhesus monkey. Radiolabeled eotaxin was used to identify a high affinity receptor on eosinophils (0.52 nM Kd), expressed at 4.8 x 10(4) sites per cell. This receptor also bound RANTES and monocyte chemotactic protein-3 with lower affinity, but not macrophage inflammatory protein-1 alpha. Eotaxin could desensitize calcium responses of eosinophils to RANTES and monocyte chemotactic protein-3, although RANTES was able to only partially desensitize eosinophil calcium responses to eotaxin. Immunohistochemistry on human nasal polyp with antieotaxin mAbs showed that certain leukocytes as well as respiratory epithelium were intensely immunoreactive, and eosinophil infiltration occurred at sites of eotaxin upregulation. Thus eotaxin in humans is a potent and selective eosinophil chemoattractant that is expressed by a variety cell types in certain inflammatory conditions.

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Anti–interleukin-5 (mepolizumab) therapy for hypereosinophilic syndromes☆

2004-01-01

Jennifer K. Garrett , Sean C. Jameson , Blythe Thomson +7 more

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Platelet-activating factor. A potent chemotactic and chemokinetic factor for human eosinophils.

1986-12-01

Andrew J. Wardlaw , Redwan Moqbel , Oliver Cromwell +1 more

Platelet-activating factor (PAF-acether), an inflammatory mediator with a wide range of biological activities including neutrophil aggregation and chemotaxis, was studied for its effect on human eosinophil locomotion (chemotaxis and chemokinesis). … Platelet-activating factor (PAF-acether), an inflammatory mediator with a wide range of biological activities including neutrophil aggregation and chemotaxis, was studied for its effect on human eosinophil locomotion (chemotaxis and chemokinesis). Human eosinophils (25-95% purity) were obtained from donors with a variety of diseases associated with hypereosinophilia. PAF-acether elicited directional locomotion of eosinophils, in a time- and dose-dependent fashion, at concentrations from 10(-5) to 10(-8) M; lyso-PAF had minimal activity over the same dose range. Compared with PAF-acether, the eosinophil locomotory responsiveness of leukotriene B4 (LTB4), histamine, and the valyl- and alanyl-eosinophil chemotactic factor of anaphylaxis (ECF-A) tetrapeptides was negligible. Conversely, neutrophil responsiveness to PAF-acether (optimum 10(-6) M) was comparable in effect to LTB4 (optimum dose 10(-8) M). It was shown that PAF-acether elicited both chemotaxis and chemokinesis of eosinophils. Comparison of normal density and light density eosinophils revealed no qualitative difference in the response to PAF-acether and the other chemoattractants, although the light density cells seemed to demonstrate a greater degree of locomotion to PAF-acether and LTB4. Thus, PAF-acether appears to be a potent eosinophilotactic agent which may play a role in inflammatory reactions characterized by eosinophil infiltration.

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An improved immunomagnetic procedure for the isolation of highly purified human blood eosinophils

1991-12-01

Trevor T. Hansel , I. Jolanda M. de Vries , Thomas Iff +5 more

SYSTEMIC LUPUS ERYTHEMATOSUS

1954-12-01

Alex Harvey , Lawrence Shulman , PHILIP A. TUMULTY +2 more

HARVEY, A. McGEHEE; SHULMAN, LAWRENCE E.; TUMULTY, PHILIP A.; CONLEY, C. LOCKARD; SCHOENRICH, EDYTH H. Author Information HARVEY, A. McGEHEE; SHULMAN, LAWRENCE E.; TUMULTY, PHILIP A.; CONLEY, C. LOCKARD; SCHOENRICH, EDYTH H. Author Information

Contemporary consensus proposal on criteria and classification of eosinophilic disorders and related syndromes

2012-03-28

Peter Valent , Amy D. Klion , Hans‐Peter Horny +7 more

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Generalized Gastrointestinal Polyposis

1955-06-16

Leonard W. Cronkhite , Wilma Jeanne Canada

TWO cases are reported below in which the presenting complaints were disturbances in gastrointestinal function, pigmentation of the skin, alopecia and atrophy of the fingernails and toenails. In each case … TWO cases are reported below in which the presenting complaints were disturbances in gastrointestinal function, pigmentation of the skin, alopecia and atrophy of the fingernails and toenails. In each case the underlying pathologic process seemed to be generalized gastrointestinal polyposis in which virtually the entire gastrointestinal mucosa was replaced by polypoid lesions. These cases are of interest in that they probably differ in nature from other syndromes that relate polyposis to pigmentation of the skin.Case ReportsCASE 1. E.S. (B.M. 733228), a 42-year-old single female bookkeeper, was first seen in March, 1951, because of nausea, vomiting and diarrhea of . . .

Two Classes of PDGF Receptor Eecognize Different Isoforms of PDGF

1988-06-10

Charles E. Hart , John W. Forstrom , James D. Kelly +5 more

Previous studies involving platelet-derived growth factor (PDGF) have been based on the premise that a single cell-surface receptor binds all three isoforms of PDGF (AA, BB, and AB). It is … Previous studies involving platelet-derived growth factor (PDGF) have been based on the premise that a single cell-surface receptor binds all three isoforms of PDGF (AA, BB, and AB). It is now shown that two populations of PDGF receptor exist and can be distinguished by their ligand binding specificity. The B receptor binds only the BB dimer, whereas the A/B receptor binds AA, BB, and AB dimers. Human dermal fibroblasts appear to express seven times as much B receptor as A/B receptor. The B receptor is responsible for most PDGF receptor phosphorylation.

Bronchoalveolar Cell Profiles of Asthmatic and Nonasthmatic Subjects

1987-08-01

John G. Kirby , Frederick E. Hargreave , Gerald J. Gleich +1 more

Asthma is associated with increased airway responsiveness to pharmacologic agents such as methacholine. Increases in airway responsiveness after exposure to allergen or ozone are associated with increased inflammatory cells in … Asthma is associated with increased airway responsiveness to pharmacologic agents such as methacholine. Increases in airway responsiveness after exposure to allergen or ozone are associated with increased inflammatory cells in bronchoalveolar lavage both in human and in animal studies. We studied the total and differential cell counts in 10 stable atopic asthmatics who had airway hyperresponsiveness but no clinical features of airway inflammation and 10 nonasthmatic subjects, using a conventional 100-ml lavage and a 20-ml washing. Metachromatic cell numbers and eosinophils were increased in both the lavage (p < 0.01 for metachromatic cells; p = 0.05 for eosinophils) and washing (p < 0.025 for metachromatic cells and p = 0.03 for eosinophils) compared with those in nonasthmatics. In asthmatics, metachromatic cell numbers in the lavage and washing, and total cell count and, to a lesser extent, eosinophils in lavage were significantly correlated with measurements of airway responsiveness. Major basic protein in lavage and blood did not differ between asthmatics and nonasthmatics. The washing, although it revealed a different cell profile from the lavage in both normal subjects and asthmatics, did not show differences between asthmatics and nonasthmatics undetected by the lavage specimen. We conclude that there is evidence of cellular inflammation in the airway of stable asthmatics and that small volume washings do not add to the information concerning the cell profile of asthmatics and nonasthmatics provided by conventional lavage.

Molecular Cloning of Human Eotaxin, an Eosinophil-selective CC Chemokine, and Identification of a Specific Eosinophil Eotaxin Receptor, CC Chemokine Receptor 3

1996-03-01

Motoji Kitaura , Toshihiro Nakajima , Toshio Imai +5 more

The CC chemokine eotaxin is a selective chemoattractant for guinea pig eosinophils, first purified from bronchoalveolar lavage fluid in a guinea pig model of allergic airway inflammation. We have now … The CC chemokine eotaxin is a selective chemoattractant for guinea pig eosinophils, first purified from bronchoalveolar lavage fluid in a guinea pig model of allergic airway inflammation. We have now isolated the gene and cDNA for a human counterpart of eotaxin. The gene maps to chromosome 17 and is expressed constitutively at high levels in small intestine and colon, and at lower levels in various other tissues. The deduced mature protein sequence is 66% identical to human monocyte chemoattractant protein-1, and 60% identical to guinea pig eotaxin. Recombinant human eotaxin produced in insect cells induced a calcium flux response in normal human eosinophils, but not in neutrophils or monocytes. The response could not be desensitized by pretreatment of eosinophils with other CC chemokines, suggesting a unique receptor. In this regard, we show that human eotaxin is a potent and highly specific agonist for CC chemokine receptor 3, a G protein-coupled receptor selectively expressed in human eosinophils. Thus eotaxin and CC chemokine receptor 3 may be host factors highly specialized for eosinophil recruitment in inflammation, and may be good targets for the development of selective drugs for inflammatory diseases where eosinophils contribute to pathogenesis, such as asthma.

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Elevated serum tryptase levels identify a subset of patients with a myeloproliferative variant of idiopathic hypereosinophilic syndrome associated with tissue fibrosis, poor prognosis, and imatinib responsiveness

2003-04-08

Amy D. Klion , Pierre Noël , Cem Akin +5 more

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The eosinophil and bronchial asthma: Current understanding

1990-02-01

Gerald J. Gleich

A human high affinity interleukin-5 receptor (IL5R) is composed of an IL5-specific α chain and a β chain shared with the receptor for GM-CSF

1991-09-01

Jan Tavernier , René Devos , Sigrid Cornelis +4 more

Association of the Eosinophilia–Myalgia Syndrome with the Ingestion of Tryptophan

1990-03-29

Philip A. Hertzman , William L. Blevins , James W. Mayer +3 more

We report on a series of three patients with an unusual syndrome of eosinophilia and myalgia associated with the oral ingestion of tryptophan that was recognized in New Mexico in … We report on a series of three patients with an unusual syndrome of eosinophilia and myalgia associated with the oral ingestion of tryptophan that was recognized in New Mexico in October 1989. All three patients, who were women 37 to 44 years of age, had severe muscle pain, muscle weakness, mouth ulcers, and striking eosinophilia (more than 8×109 cells per liter). Other manifestations included fever, abdominal pain, dyspnea, skin rash, and elevated serum concentrations of aminotransferase and aldolase.

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Messenger RNA expression of the cytokine gene cluster, interleukin 3 (IL-3), IL-4, IL-5, and granulocyte/macrophage colony-stimulating factor, in allergen-induced late-phase cutaneous reactions in atopic subjects.

1991-03-01

A B Kay , Sun Ying , Veronica Varney +5 more

Cryostat sections from skin biopsies from 24-h allergen-induced late-phase cutaneous reactions (LPR) in 14 human atopic subjects were hybridized with 35S-labeled RNA probes for a number of cytokines. mRNA was … Cryostat sections from skin biopsies from 24-h allergen-induced late-phase cutaneous reactions (LPR) in 14 human atopic subjects were hybridized with 35S-labeled RNA probes for a number of cytokines. mRNA was detected for interleukin 3 (IL-3) (8/14), IL-4 (10/14), IL-5 (11/14), and granulocyte/macrophage colony-stimulating factor (GM-CSF) (13/14). Only 5 of 14 gave hybridization signals for IL-2, and 0 of 14 for interferon gamma. Biopsies from diluent controls gave only occasional weak signals. These results suggest that cells infiltrating the site of the 24-h LPR transcribe mRNA for the IL-3, IL-4, IL-5, and GM-CSF gene cluster and support the hypothesis that atopy is associated with preferential activation of cells having a similar cytokine profile to the murine T helper type 2 subset.

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Mechanism of Action and In Vivo Role of Platelet-Derived Growth Factor

1999-01-10

Carl‐Henrik Heldin , Bengt Westermark

Platelet-derived growth factor (PDGF) is a major mitogen for connective tissue cells and certain other cell types. It is a dimeric molecule consisting of disulfide-bonded, structurally similar A- and B-polypeptide … Platelet-derived growth factor (PDGF) is a major mitogen for connective tissue cells and certain other cell types. It is a dimeric molecule consisting of disulfide-bonded, structurally similar A- and B-polypeptide chains, which combine to homo- and heterodimers. The PDGF isoforms exert their cellular effects by binding to and activating two structurally related protein tyrosine kinase receptors, denoted the α-receptor and the β-receptor. Activation of PDGF receptors leads to stimulation of cell growth, but also to changes in cell shape and motility; PDGF induces reorganization of the actin filament system and stimulates chemotaxis, i.e., a directed cell movement toward a gradient of PDGF. In vivo, PDGF has important roles during the embryonic development as well as during wound healing. Moreover, overactivity of PDGF has been implicated in several pathological conditions. The sis oncogene of simian sarcoma virus (SSV) is related to the B-chain of PDGF, and SSV transformation involves autocrine stimulation by a PDGF-like molecule. Similarly, overproduction of PDGF may be involved in autocrine and paracrine growth stimulation of human tumors. Overactivity of PDGF has, in addition, been implicated in nonmalignant conditions characterized by an increased cell proliferation, such as atherosclerosis and fibrotic conditions. This review discusses structural and functional properties of PDGF and PDGF receptors, the mechanism whereby PDGF exerts its cellular effects, and the role of PDGF in normal and diseased tissues.

CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy

2003-07-08

Animesh Pardanani , Rhett P. Ketterling , Stephanie R. Brockman +7 more

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Molecular cloning and characterization of a human eotaxin receptor expressed selectively on eosinophils.

1996-06-01

Paul Ponath , Suofu Qin , T W Post +6 more

The chemokine eotaxin is unusual in that it appears to be a highly specific chemoattractant for eosinophils. Ligand-binding studies with radiolabeled eotaxin demonstrated a receptor on eosinophils distinct from the … The chemokine eotaxin is unusual in that it appears to be a highly specific chemoattractant for eosinophils. Ligand-binding studies with radiolabeled eotaxin demonstrated a receptor on eosinophils distinct from the known chemokine receptors CKR-1 and -2. The distinct eotaxin binding site on human eosinophils also bound RANTES (regulated on activation T expressed and secreted) and monocyte chemotactic protein (MCP)3. We have now isolated a cDNA from eosinophils, termed CKR-3, with significant sequence similarity to other well characterized chemokine receptors. Cells transfected with CKR-3 cDNA bound radiolabeled eotaxin specifically and with high affinity, comparable to the binding affinity observed with eosinophils. This receptor also bound RANTES and MCP-3 with high affinity, but not other CC or CXC chemokines. Furthermore, receptor transfectants generated in a murine B cell lymphoma cell line migrated in transwell chemotaxis assays to eotaxin, RANTES, and MCP-3, but not to any other chemokines. A monoclonal antibody recognizing CKR-3 was used to show that eosinophils, but not other leukocyte types, expressed this receptor. This pattern of expression was confirmed by Northern blot with RNA from highly purified leukocyte subsets. The restricted expression of CKR-3 on eosinophils and the fidelity of eotaxin binding to CKR-3, provides a potential mechanism for the selective recruitment and migration of eosinophils within tissues.

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PDGF receptors as cancer drug targets

2003-05-01

Kristian Pietras , Tobias Sjöblom , Kristofer Rubin +2 more

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Nephrogenic Fibrosing Dermopathy

2001-10-01

Shawn E. Cowper , Lyndon D. Su , Jag Bhawan +2 more

This report details the histopathologic findings in a unique fibrosing disorder that recently emerged among patients with renal disease. The affected patients were initially identified among recipients of renal transplants … This report details the histopathologic findings in a unique fibrosing disorder that recently emerged among patients with renal disease. The affected patients were initially identified among recipients of renal transplants at a single institution, but later cases at other centers were identified, and included patients receiving renal dialysis for a variety of different kidney diseases. The cutaneous changes consisted largely of indurated plaques and papules on the extremities and trunk. Systemic findings seen in scleromyxedema, which the condition resembles in some respects, were absent. By routine microscopy, the findings range from a very subtle proliferation of dermal fibroblasts in early lesions, to a florid proliferation of fibroblasts and dendritic cells in fully developed cases. Thick collagen bundles with surrounding clefts are a prominent finding, and a variable increase in dermal mucin and elastic fibers was usually evident with special stains. CD-34 positive dermal dendrocytes were floridly abundant, with dendritic processes aligned with elastic fibers and around collagen bundles in a dense network. Factor XIIIa and CD-68 positive mono-and multinucleated cells are also present in increased numbers. Electron microscopy highlighted increased elastic fibers closely apposed to dendritic cell processes. The entire dermis was commonly involved, with increased spindle cells, collagen, mucin, and elastic fibers extending through the subcutis along the septa of fatty lobules. In some instances, the process resembled a sarcoma on histopathologic examination. The recent emergence of this condition and the apparent clustering of cases in specific dialysis centers initially suggested a possible infectious and/or toxic agent. To date, however, no such agent has been identified. We propose the term "nephrogenic fibrosing dermopathy (NFD)" until a specific cause can be identified.

IL-5- and eosinophil-mediated inflammation: from discovery to therapy

2009-10-09

Taku Kouro , Kiyoshi Takatsu

IL-5 was originally defined as a T-cell-derived cytokine that triggers activated B cells for terminal differentiation into antibody-secreting plasma cells, at least in mice. Concurrently, IL-5 was recognized as the … IL-5 was originally defined as a T-cell-derived cytokine that triggers activated B cells for terminal differentiation into antibody-secreting plasma cells, at least in mice. Concurrently, IL-5 was recognized as the major maturation and differentiation factor for eosinophils in mice and humans. Over-expression of IL-5 significantly increases eosinophil numbers and antibody levels in vivo. Conversely, mice lacking a functional gene for IL-5 or the IL-5 receptor alpha chain (IL-5Rα) display a number of developmental and functional impairments in B-cell and eosinophil lineages. In addition to the Janus kinase–signal transducer and activator of transcription pathway, the tyrosine kinases Lyn and Btk (Bruton agammaglobulinemia tyrosine kinase) are involved, and Ras GTPase–extracellular signal-regulated kinase (Ras–ERK) signals are important for IL-5-dependent cell proliferation and survival. IL-5 critically regulates expression of genes involved in proliferation, cell survival and maturation and effector functions of B cells and eosinophils. Thus, IL-5 plays a pivotal role in innate and acquired immune responses and eosinophilia. In humans, the biologic effects of IL-5 are best characterized for eosinophils. The recent expansion in our understanding of the mechanisms of eosinophil development and activation in the context of IL-5 has led to advances in therapeutic options. A new therapy currently in clinical trials uses humanized mAbs against IL-5 or the IL-5R.

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Eosinophilic fasciitis: Clinical spectrum and therapeutic response in 52 cases

1988-05-01

Sharad Lakhanpal , William W. Ginsburg , Clement J. Michet +2 more

Treatment of Patients with the Hypereosinophilic Syndrome with Mepolizumab

2008-03-17

Marc E. Rothenberg , Amy D. Klion , Florence Roufosse +7 more

The hypereosinophilic syndrome is a group of diseases characterized by persistent blood eosinophilia, defined as more than 1500 cells per microliter with end-organ involvement and no recognized secondary cause. Although … The hypereosinophilic syndrome is a group of diseases characterized by persistent blood eosinophilia, defined as more than 1500 cells per microliter with end-organ involvement and no recognized secondary cause. Although most patients have a response to corticosteroids, side effects are common and can lead to considerable morbidity.We conducted an international, randomized, double-blind, placebo-controlled trial evaluating the safety and efficacy of an anti-interleukin-5 monoclonal antibody, mepolizumab, in patients with the hypereosinophilic syndrome. Patients were negative for the FIP1L1-PDGFRA fusion gene and required prednisone monotherapy, 20 to 60 mg per day, to maintain a stable clinical status and a blood eosinophil count of less than 1000 per microliter. Patients received either intravenous mepolizumab or placebo while the prednisone dose was tapered. The primary end point was the reduction of the prednisone dose to 10 mg or less per day for 8 or more consecutive weeks.The primary end point was reached in 84% of patients in the mepolizumab group, as compared with 43% of patients in the placebo group (hazard ratio, 2.90; 95% confidence interval [CI], 1.59 to 5.26; P<0.001) with no increase in clinical activity of the hypereosinophilic syndrome. A blood eosinophil count of less than 600 per microliter for 8 or more consecutive weeks was achieved in 95% of patients receiving mepolizumab, as compared with 45% of patients receiving placebo (hazard ratio, 3.53; 95% CI, 1.94 to 6.45; P<0.001). Serious adverse events occurred in seven patients receiving mepolizumab (14 events, including one death; mean [+/-SD] duration of exposure, 6.7+/-1.9 months) and in five patients receiving placebo (7 events; mean duration of exposure, 4.3+/-2.6 months).Our study shows that treatment with mepolizumab, an agent designed to target eosinophils, can result in corticosteroid-sparing for patients negative for FIP1L1-PDGFRA who have the hypereosinophilic syndrome. (ClinicalTrials.gov number, NCT00086658 [ClinicalTrials.gov].).

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Pharmacology of the Eosinophil

1999-06-01

Mark A. Giembycz , Mark A. Lindsay

The Immunobiology of Eosinophils

1991-04-18

Jeffrey S. Flier , Lisa H. Underhill , Peter F. Weller

EOSINOPHILS, like neutrophils and basophils, are a type of granulocyte derived from bone marrow, distinguished by their morphologic features, constituents, products, and associations with specific diseases. The original denominating property … EOSINOPHILS, like neutrophils and basophils, are a type of granulocyte derived from bone marrow, distinguished by their morphologic features, constituents, products, and associations with specific diseases. The original denominating property of eosinophils was the cardinal affinity of their cytoplasmic granules for acid aniline dyes, such as eosin. Eosinophils contain several eosinophil-specific proteins in their cytoplasmic granules, yet to date no cell-surface proteins unique to eosinophils have been recognized. Thus, tinctorial properties remain the routine basis for identifying and enumerating these leukocytes in blood and tissues. Eosinophilia, characterized by both heightened production of eosinophils in bone marrow and the accumulation of . . .

Episodic Angioedema Associated with Eosinophilia

1984-06-21

Gerald J. Gleich , Arnold L. Schroeter , J. Paul Marcoux +3 more

Four patients with recurrent attacks of angioedema, urticaria, and fever were seen. During attacks, body weights increased up to 18% and leukocyte counts reached 108,000/microliters (88% eosinophils). Glucocorticoid therapy caused … Four patients with recurrent attacks of angioedema, urticaria, and fever were seen. During attacks, body weights increased up to 18% and leukocyte counts reached 108,000/microliters (88% eosinophils). Glucocorticoid therapy caused defervescence, diuresis, and decreased total leukocyte and eosinophil counts. The two children received prednisone intermittently; the adults did not require treatment or their conditions were controlled by alternate-day prednisone administration. No patient had evidence of cardiac involvement (follow-up, 2-17 years). The disease does not threaten the function of vital organs. One patient remained in spontaneous remission for 20 years before symptoms recurred. Although it might be classified as a variant of the hypereosinophilic syndrome, we believe that this syndrome is a separate entity because of its distinctive characteristics and its benign course.

Total Eosinophil Counts in the Management of Bronchial Asthma

1975-05-29

Barry R. Horn , Eugene D. Robin , James Theodore +1 more

Total eosinophil counts were investigated in asthmatic patients to determine their usefulness in the diagnosis and management of steroid-dependent asthma. Counts averaged 122 plus or minus 74 (S.D.) per mm-3 … Total eosinophil counts were investigated in asthmatic patients to determine their usefulness in the diagnosis and management of steroid-dependent asthma. Counts averaged 122 plus or minus 74 (S.D.) per mm-3 (65 untreated normal subjects) and 43 plus or minus 22 per mm-3 (six prednisone-treated normal subjects). Fifty-two patients with active bronchial asthma showed significant eosinophilia (greater than 350/mm-3 off and greater than 85/mm-3 on steroids), suggesting that eosinophilia is an important diagnostic feature of bronchial asthma. In 14 patients (60 observations), the counts showed significant inverse correlation with specific airway conductance--r equals 0.74, p less than 0.001--and with a variety of other measurements of bronchial dynamics and lung volumes, suggesting that the total eosinophil count reflects asthmatic activity and is useful for regulating steroid dosage and for early detection of exacerbations.

Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis

2017-05-17

Michael E. Wechsler , Praveen Akuthota , David Jayne +7 more

Eosinophilic granulomatosis with polyangiitis is an eosinophilic vasculitis. Mepolizumab, an anti–interleukin-5 monoclonal antibody, reduces blood eosinophil counts and may have value in the treatment of eosinophilic granulomatosis with polyangiitis. Eosinophilic granulomatosis with polyangiitis is an eosinophilic vasculitis. Mepolizumab, an anti–interleukin-5 monoclonal antibody, reduces blood eosinophil counts and may have value in the treatment of eosinophilic granulomatosis with polyangiitis.

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Abnormal Clones of T Cells Producing Interleukin-5 in Idiopathic Eosinophilia

1999-10-07

Hans‐Uwe Simon , Sabine G. Plötz , Reinhard Dummer +1 more

The cause of persistent eosinophilia and the hypereosinophilic syndrome is unknown. Recent work suggests that in some patients with the hypereosinophilic syndrome, a clone of abnormal T cells produces large … The cause of persistent eosinophilia and the hypereosinophilic syndrome is unknown. Recent work suggests that in some patients with the hypereosinophilic syndrome, a clone of abnormal T cells produces large amounts of interleukin-5, a cytokine required for the growth and differentiation of eosinophils. We examined T-cell surface markers, rearranged T-cell–receptor genes, and in vitro production of cytokines by T cells from patients with idiopathic eosinophilia.

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Eosinophilia

1998-05-28

Marc E. Rothenberg

A marked accumulation of eosinophils occurs in several important disorders, such as allergic diseases, parasitic infections, and cancer.1 The level of eosinophils in the body is normally tightly regulated. In … A marked accumulation of eosinophils occurs in several important disorders, such as allergic diseases, parasitic infections, and cancer.1 The level of eosinophils in the body is normally tightly regulated. In normal subjects, eosinophils account for only a small minority of peripheral-blood leukocytes, and their presence in tissues is primarily limited to the gastrointestinal mucosa.2 In certain disease states, however, eosinophils can selectively accumulate in the peripheral blood or any tissue in the body. Any perturbation that results in eosinophilia, defined here as an abnormal accumulation of eosinophils in blood or tissue, can have profound clinical effects. Eosinophilia may be harmful, . . .

Interleukin-5, eosinophils, and disease

1992-06-15

CJ Sanderson

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Evaluating the efficacy and safety of benralizumab in eosinophilic granulomatosis with polyangiitis: a review of clinical trials versus real-world evidence

2025-06-25

Ikponmwosa Jude Ogieuhi , Chidera Stanley Anthony , Victor Oluwatomiwa Ajekiigbe +7 more | Deleted Journal

Papulosquamous Rash Progressing to Erythroderma With Worsening Eosinophilia: Challenge

2025-06-25

Yuta Kioi , Kohei Ogawa , Satoru Shinkuma +4 more | American Journal of Dermatopathology

Forearm Pain and Stiffness in a 34-Year-Old Woman

2025-06-25

Jamila Mammadova , Tracy Frech , Erin Chew | JAMA

A 34-year-old woman had progressive skin tightening over her right elbow and forearm, bilateral forearm pain, difficulty extending her fingers and elbows, and elevated eosinophil count and C-reactive protein level; … A 34-year-old woman had progressive skin tightening over her right elbow and forearm, bilateral forearm pain, difficulty extending her fingers and elbows, and elevated eosinophil count and C-reactive protein level; examination showed a linear depression along a superficial vein when the right forearm was raised. What is the diagnosis and what would you do next?

Primary care physicians play a crucial role in diagnosing and managing rare eosinophilic diseases: HES and EGPA

2025-06-24

Mili Shum , Ora Gewurz‐Singer , Jared Silver +1 more | Frontiers in Medicine

Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) are chronic eosinophilic diseases with serious multisystem manifestations. Patients with HES or EGPA often fail to receive a timely diagnosis, and … Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) are chronic eosinophilic diseases with serious multisystem manifestations. Patients with HES or EGPA often fail to receive a timely diagnosis, and while these conditions are considered rare, frequent under-recognition indicates that their true prevalence likely exceeds current estimates. Increased primary care physician (PCP) awareness of these systemic eosinophilic conditions and the “red flags” that should trigger referral will help more patients receive timely diagnosis and care. Patients with HES or EGPA present with a heterogeneous range of symptoms and manifestations that can overlap with other conditions, making diagnosis challenging. PCPs should be aware that the following are red flags that warrant further investigation and trigger expert referral: blood eosinophil count ≥10% of total peripheral white blood cells or ≥1,000 cells/μL; persistent hypereosinophilia, noting that systemic corticosteroid treatment may variably impact the degree of eosinophilia; refractory asthma symptoms with the need for prolonged or recurrent systemic corticosteroid treatment; reports of decreasing efficacy to asthma therapy; extra-pulmonary findings in the setting of eosinophilia; multiorgan system involvement; and evolving or worsening signs and symptoms over periods of weeks to months or years. PCPs play a key role in the diagnosis and management of rare eosinophilic diseases. By being aware of HES and EGPA and their associated red flags, PCPs are well-placed to recognize these conditions early, trigger further investigations, and coordinate effective multidisciplinary care. This can help patients receive a more accurate diagnosis on time and faster access to treatment, which may ultimately improve patient outcomes.

A case of angioimmunoblastic T-cell lymphoma mimicking angioedema

2025-06-23

He Zhao , Xia Yun , Fei Su +2 more | Discover Oncology

Erythema Multiforme-Like Lesions Revealing Systemic Lupus Erythematosus

2025-06-23

Hanae Tassine , Abire Allaoui , Abdelhamid Naitlhou | Cureus

Clinical Presentation and Diagnosis of Multisystemic Eosinophilic Epitheliotropic Disease in a Miniature Donkey: A Case Report

2025-06-23

Carla Katherine Enriquez , Alicia E. Long , Cristobal Navas de Solís +2 more | Journal of Veterinary Internal Medicine

ABSTRACT A 21‐year‐old miniature donkey gelding was evaluated for pruritus, inappetence, hypertriglyceridemia, and alopecia of 2 weeks' duration. Hematology showed moderate eosinophilia, severe hypertriglyceridemia, and moderately increased liver enzyme activities. … ABSTRACT A 21‐year‐old miniature donkey gelding was evaluated for pruritus, inappetence, hypertriglyceridemia, and alopecia of 2 weeks' duration. Hematology showed moderate eosinophilia, severe hypertriglyceridemia, and moderately increased liver enzyme activities. Cytologic evaluation of peritoneal fluid identified an eosinophilic transudate. The donkey was treated with antihistamines, anthelmintics, IV fluids with dextrose as a constant rate infusion, and insulin. The miniature donkey was euthanized because of the development of laminitis. Lesions associated with multisystemic eosinophilic epitheliotropic disease (MEED) were identified at necropsy.

Carmellose/Prednisolone

2025-06-21

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Busulfan/cyclophosphamide/etoposide

2025-06-21

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Diffuse Alveolar Hemorrhage as the First Presentation of Systemic Lupus Erythematosus: A Case Report.

2025-06-21

Selma Later , Muzan Abdelbagi , Amjad Zaaroura +4 more | PubMed

BACKGROUND Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening complication of systemic lupus erythematosus (SLE), with a mortality rate of up to 80%. It is seldom the initial manifestation … BACKGROUND Diffuse alveolar hemorrhage (DAH) is a rare but life-threatening complication of systemic lupus erythematosus (SLE), with a mortality rate of up to 80%. It is seldom the initial manifestation of SLE, making early diagnosis and treatment challenging. DAH typically presents with hemoptysis, abrupt anemia, and diffuse pulmonary infiltrates, although up to 50% of cases lack hemoptysis, complicating diagnosis. CASE REPORT We present a case of a 23-year-old woman with no prior SLE diagnosis who presented with dyspnea, cough, and fever. Initial investigations revealed normocytic normochromic anemia (hemoglobin 7.3 g/dL), acute kidney injury (creatinine 323 µmol/L), and bilateral pulmonary infiltrates on imaging. DAH was confirmed via bronchoscopy, which showed hemorrhagic bronchoalveolar lavage fluid, and high-resolution computed tomography of the chest revealed pulmonary consolidations with peripheral sparing. Autoimmune workup confirmed SLE, with positive antinuclear antibodies, anti-dsDNA antibodies, and low complement levels. The patient was treated with high-dose corticosteroids, rituximab, and plasma exchange. Despite initial stabilization and weaning from mechanical ventilation, she developed refractory pancytopenia, posterior reversible encephalopathy syndrome, and multi-organ failure, leading to her death on hospital day 48. CONCLUSIONS This case stresses the value of a high index of suspicion for DAH in young women with respiratory symptoms, even in the absence of a known SLE diagnosis. Early diagnostic measures, including bronchoscopy and imaging, are critical for timely intervention. Aggressive immunosuppressive therapy and plasma exchange can stabilize patients temporarily, but refractory cases remain challenging. A multidisciplinary approach is essential to improve outcomes in this high-mortality condition.

Cobimetinib/peginterferon-alfa

2025-06-21

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Enoxaparin sodium/Letrozole

2025-06-21

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Prednisolone/Propranolol

2025-06-21

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Fascitis eosinofílica

2025-06-20

Iago Pinal‐Fernandez , José C. Milisenda , Albert Selva-O’Callaghan | Medicina Clínica

Eosinophilic myocarditis due to parasitic infection: A case-based minireview

2025-06-20

Thang Viet Luong , Tiến Hoàng Anh , Ngoc-Thac Pham +4 more | World Journal of Cardiology

Eosinophilic myocarditis (EM) is a rare inflammatory condition of the heart, often associated with eosinophilic infiltration. While its causes range from allergies to autoimmune and infectious diseases, parasitic infections are … Eosinophilic myocarditis (EM) is a rare inflammatory condition of the heart, often associated with eosinophilic infiltration. While its causes range from allergies to autoimmune and infectious diseases, parasitic infections are an uncommon but critical etiology. This mini-review focuses on a case of EM in a 47-year-old male from Vietnam, linked to Schistosoma spp., Strongyloides stercoralis, and Toxocara spp. infections. The patient presented with severe chest pain and recovered fully after treatment with corticosteroids and albendazole. Drawing insights from this case and existing literature, we discuss the pathophysiology, diagnostic approaches, and therapeutic strategies for parasite-induced EM. Early diagnosis and tailored treatment are essential to improve clinical outcomes, especially in endemic parasitic areas.

Peripheral Neuropathy Presenting With Hypereosinophilia: A Quiz

2025-06-19

Rex K. Siu , Amanda Hardy , Faaiq Aslam +5 more | American Journal of Kidney Diseases

CYTOLOGICAL DIAGNOSIS AND SUCCESSFUL TREATMENT OF INDOLENT ULCER IN A PERSIAN CAT: A CASE REPORT

2025-06-19

B.K. Bhagya , Venkataram Shivakumar , K. Kavitha +1 more | Indian journal of veterinary and animal sciences research.

A one year old, female, Persian cat, weighing about 4kg was presented to Veterinary College Hospital, Hassan with a history of swollen upper lip and presence of ulcers since one … A one year old, female, Persian cat, weighing about 4kg was presented to Veterinary College Hospital, Hassan with a history of swollen upper lip and presence of ulcers since one week. The cat was not showing any signs of pruritus and was taking food normally. Physical examination revealed swollen upper lip. Impression smear of ulcerative lesion revealed presence of predominant number of eosinophils, ruptured eosinophils leaving naked nuclei, eosinophilic granules in background and infiltration of neutrophils in some areas. Based on history, clinical signs and cytology, the case was diagnosed as eosinophilic ulcer (indolent ulcer). The animal was managed successfully with glucocorticoids and antibiotics.

A 29-Year-Old Male with Eosinophilic Myocarditis and Multisystem Involvement: A Diagnostic Dilemma Between EGPA and Idiopathic Hypereosinophilic Syndrome

2025-06-18

Shan Ma , Yanqing Hu , Shuaiye Liu +4 more | Next research.

High Disease Burden and Oral Corticosteroid Use in Patients with Hypereosinophilic Syndrome and Eosinophilic Granulomatosis with Polyangiitis: Country-Level Insights into Real-World Management in Europe

2025-06-17

Jeremiah Hwee , Lynn Huynh , Thanai Pongdee +4 more | Journal of Clinical Medicine

Objectives: To analyze variations in patient characteristics, treatment patterns, clinical manifestations, clinical outcomes (i.e., response, flares and flare-free survival in HES; remission, relapses and relapse-free survival in EGPA; and overall … Objectives: To analyze variations in patient characteristics, treatment patterns, clinical manifestations, clinical outcomes (i.e., response, flares and flare-free survival in HES; remission, relapses and relapse-free survival in EGPA; and overall survival), and healthcare resource utilization (HCRU) in patients with hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) across five European countries. Methods: In two parallel, retrospective, non-interventional, longitudinal chart review studies (GSK ID: 214661 [EGPA] and 214657 [HES]), physicians collected data of patients they treated in France, Germany, Italy, Spain, and the UK from January 2015 to December 2019, with follow-up until August 2021. Country-level results are presented for each study; HES and EGPA data were pooled in a post hoc exploratory analysis. Results: Per-country, 22-26 HES- and 38-45 EGPA-treating physicians collected data from 52-62 (total 280) patients with HES and 80-85 (total 407) with EGPA. Patient sex and age at diagnosis differed across countries. Pooled HES/EGPA data revealed high oral corticosteroid (OCS) use in all countries (94.9% of patients; median [IQR] duration 20.7 [9.0, 33.8] months); immunosuppressive treatments and biologics use varied between countries (43.7-61.5% and 25.6-59.8%, respectively). The most frequent clinical manifestations were constitutional (51.6-78.8%) and lung (43.5-55.8%) in HES, and lung (41.3-67.9) and ENT (43.8-61.2) in EGPA. Pooled HCRU data showed country-level variation; 70.7-91.2% of patients had disease-related outpatient visits and 36.1-52.6% had ER visits or hospitalizations. Conclusions: Results demonstrate substantial disease burden, including high HCRU and extensive OCS use among patients with HES and EGPA in five European countries. The findings highlight the need for improved treatment strategies such as optimizing use of biologics to mitigate the reliance on corticosteroids.

Rothmund-Thomson Syndrome Type II: A Pediatric Case Presentation with Genetic and Dermoscopic Findings

2025-06-14

Loubaris Zineb , Moumna Rasha , Benzekri Laila +1 more | Asian Journal of Pediatric Research

Background: Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis characterized by poikiloderma, sparse hair, skeletal anomalies, and an increased risk of malignancies, particularly osteosarcoma and skin cancers. RTS is … Background: Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis characterized by poikiloderma, sparse hair, skeletal anomalies, and an increased risk of malignancies, particularly osteosarcoma and skin cancers. RTS is classified into two types: Type I with an unknown genetic cause and Type II associated with mutations in the RECQL4 gene. Case Report: We report the case of an 11-year-old male born to first-degree consanguineous parents, who presented with coarse, sparse hair and erythematous facial lesions since infancy, evolving into hypo- and hyperpigmented macules. Clinical examination revealed poikiloderma affecting the hands, Gluteal area, and feet, with skin atrophy, telangiectasias, eyebrow and eyelash alopecia, plantar hyperkeratosis, thumb hypoplasia, and psychomotor developmental delay. Dermoscopic evaluation demonstrated heterogeneous pigmentation with fine arborizing telangiectasias, without malignant features. Genetic analysis identified a pathogenic c.2335_2356del, p.(Asp779Cysfs*57) mutation in the RECQL4 gene, confirming the diagnosis of RTS Type II. Conclusion: This case highlights the importance of considering RTS in pediatric patients presenting with poikiloderma and developmental anomalies, particularly in consanguineous populations. Genetic confirmation is essential for accurate diagnosis, and long-term surveillance is crucial due to the increased risk of malignancies associated with RTS Type II.

Reversible Dementia With Hypereosinophilia: An Intricate Connection

2025-06-14

Ajay Emani , Ramakant Yadav , Roopesh Kirar +1 more | Cureus

Hypereosinophilic syndrome (HES) is a rare group of disorders characterized by elevated eosinophil levels, leading to tissue infiltration and damage. Neurological complications are observed in over half of the patients. … Hypereosinophilic syndrome (HES) is a rare group of disorders characterized by elevated eosinophil levels, leading to tissue infiltration and damage. Neurological complications are observed in over half of the patients. This case study discusses a 45-year-old male patient diagnosed with HES and rapidly progressive dementia. The patient presented with difficulties in routine tasks, attention deficits, memory loss, and frontal headaches lasting one year. His medical history included allergic rhinitis and eczematoid skin lesions. General and neurological examinations revealed deficits in higher mental functions without other abnormalities. Laboratory tests showed significant eosinophilia, and magnetic resonance imaging (MRI) of the brain indicated diffuse cortical atrophy and periventricular hyperintensities. The diagnosis of HES was supported by clinical and laboratory findings. The case highlights the importance of considering HES in patients with unexplained neurological symptoms and eosinophilia to prevent irreversible organ damage. Early recognition and appropriate management are crucial for improving patient outcomes. This study underscores the need for further research to understand HES's pathophysiology and develop targeted therapies.

Child With Painful Purpuric Rash of the Hands and Feet

2025-06-12

Andrew J. Gauger , Henry E. Jones , Simon Warren +1 more | Pediatric Dermatology

Optimizing diagnostic and management pathways for patients with eosinophilia of unknown origin: a multidisciplinary protocol for urgent and non-urgent evaluation

2025-06-04

Silvia Sánchez‐Ramón , Gloria Candelas , José‐Ángel Hernández‐Rivas +7 more | Frontiers in Medicine

Introduction Persistent eosinophilia of unknown cause is a key feature of numerous health disorders. These conditions present diagnostic and management challenges, often leading to delayed treatment, increased morbidity and mortality … Introduction Persistent eosinophilia of unknown cause is a key feature of numerous health disorders. These conditions present diagnostic and management challenges, often leading to delayed treatment, increased morbidity and mortality and unnecessary healthcare costs. A systematic approach to patient flow can streamline the process from presentation with eosinophilia to triage management, in hospital settings. Methods A proposal of a novel patient flow pathway was developed through a collaborative effort involving 15 diverse multidisciplinary specialists in a public-funded tertiary teaching hospital located in Madrid, Spain, for managing eosinophilic diseases. This pathway focuses on early identification and expedited referral circuits in severe cases of hypereosinophilia and early screening of primary immunodeficient patients to optimize the journey from initial presentation through diagnosis and initial management. Results The proposed patient flow model is designed to be replicable in other hospital settings. Its implementation aims to facilitate timely diagnosis and reduce the preventable morbidity, mortality, suffering and economic burden associated with complex eosinophilic conditions. Conclusion The development and implementation of a structured patient flow pathway for eosinophilia of unknown cause in a tertiary hospital setting demonstrates a significant step toward improving patient outcomes. This model serves as a template for other healthcare institutions seeking to enhance the management and care of patients with eosinophilic diseases.

Anti-neutrophil cytoplasm antibody-negative eosinophilic granulomatous polyangiitis complicated by myositis: a case report

2025-06-02

Naoki Nakagawa , Eiichi Kakehi , Kazuhiko Kotani | Modern Rheumatology Case Reports

Abstract A 31-year-old woman visited our hospital with swelling and pain in both forearms of 2 months’ duration, followed by swelling and pain in both thighs. Her medical history included … Abstract A 31-year-old woman visited our hospital with swelling and pain in both forearms of 2 months’ duration, followed by swelling and pain in both thighs. Her medical history included bronchial asthma at the age of 18 years. After the birth of her first child at 30 years of age, her asthma worsened, and was accompanied by abdominal pain and skin rash, with no identifiable cause. Blood testing showed eosinophilia and high muscle enzyme activities, but she was anti-neutrophilic cytoplasmic antibody (ANCA)-negative. Magnetic resonance imaging of her forearms and thighs revealed strong signals on T2-weighted images in the fascia and muscle. Skin-to-muscle en bloc biopsy showed eosinophilic infiltration of muscle and small vessels. She was diagnosed with eosinophilic granulomatosis with polyangiitis (EGPA), complicated by myositis, although EGPA is usually accompanied by ANCA-positivity in approximately half of cases. Treatment was started with prednisolone alone at 0.5 mg/kg/day, and her symptoms and eosinophil count quickly improved. Clinicians should note the possibility of ANCA-negative EGPA complicated by myositis.

POS0326 EFFICACY OF TWO YEARS OF TREATMENT WITH ANTI-IL-5/R THERAPY FOR ACHIEVING GLUCOCORTICOID-FREE SUSTAINED REMISSION IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS

2025-06-01

P. A. Merkel , Nancy Agmon‐Levin , David J. Jackson +7 more | Annals of the Rheumatic Diseases

OP0170 LONG-TERM TREATMENT WITH LOW-DOSE MEPOLIZUMAB FOR EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS: FOLLOW-UP RESULTS FROM THE EUROPEAN EGPA STUDY GROUP

2025-06-01

Alessandra Bettiol , Irene Mattioli , L. Vrola +7 more | Annals of the Rheumatic Diseases

Idiopathic Hypereosinophilic Syndrome Presenting with Eosinophilic Pleural and Pericardial Effusions: A Case Report

2025-06-01

Abdul Qadir , Mamunul Islam , Riyadh Ali Mohammed Hammamy +2 more | Medical Reports

POS0560 Diagnostic Yield of Tissue Biopsies in Eosinophilic Granulomatosis with Polyangiitis (EGPA): A Monocentric Retrospective Study

2025-06-01

Francesca Mascia , Giovanni Bertalot , F. Ravanelli +3 more | Annals of the Rheumatic Diseases

Eosinophils and other blood cell traits in the risk of infertility population: A Mendelian randomization analysis

2025-06-01

Shiqi Yi , Ying Feng , Yuping Zhang +5 more | Journal of Obstetrics and Gynaecology Research

Abstract Background Abnormalities in blood cell parameters, including eosinophil traits, have been implicated in reproductive system damage. However, their specific relationship with infertility and abnormal spermatozoa remains unclear. Objective In … Abstract Background Abnormalities in blood cell parameters, including eosinophil traits, have been implicated in reproductive system damage. However, their specific relationship with infertility and abnormal spermatozoa remains unclear. Objective In this study, we investigated 23 blood cell parameters, including eosinophil traits, using bidirectional Mendelian randomization analysis to explore their causal relationship with male and female infertility. Methods We utilized data from the INTERVAL study, UK Biobank, Blood Cell Consortium, and GWAS Catalog for 23 blood cell parameters, as well as GWAS data from the FinnGen database for two infertile populations and one clinical phenotype related to infertility. MR analysis was conducted employing various methods, including inverse‐variance weighting (IVW), to obtain statistically significant results. Results Among the blood cell parameters examined, three were associated with the risk of male infertility: eosinophil percentage of granulocytes, eosinophil cell count, and mean sphered cell volume. Among these, eosinophil levels exhibited the most significant correlation with male infertility. In contrast, no blood cell traits were identified as risk factors for female infertility. However, basophil cell count and hemoglobin concentration‐related traits appeared to serve as protective factors for female infertility. Conclusion Abnormal eosinophil levels may increase the risk of male infertility. However, given the limitations inherent in the observational design of this MR study, further research is required to assess the potential therapeutic effects of eosinophil reduction in idiopathic male infertility. Regarding female infertility, the study suggests that certain blood cell traits, such as basophil cell count, may act as protective factors, which warrants further investigation.

The Use of Optical Genome Mapping for the Detection of Tyrosine Kinase Gene Fusions in Myeloid/Lymphoid Neoplasms

2025-06-01

Justine Vanhevel , Katrina Rack , Geneviève Ameye +4 more | Journal of Cellular and Molecular Medicine

ABSTRACT Myeloid/Lymphoid Neoplasms with eosinophilia and involvement of Tyrosine Kinase gene fusions (MLN‐TK) is a WHO disease category including a diverse group of malignancies characterised by recurrent genomic rearrangements of … ABSTRACT Myeloid/Lymphoid Neoplasms with eosinophilia and involvement of Tyrosine Kinase gene fusions (MLN‐TK) is a WHO disease category including a diverse group of malignancies characterised by recurrent genomic rearrangements of tyrosine kinase (TK) genes such as PDGFRA , PDGFRB , FGFR1 , JAK2 , ETV6 and FLT3 . Identification of these TK rearrangements is important for the accurate diagnosis of MLN‐TK and allows targeted therapy with TK inhibitors. In this study, we validated the use of optical genome mapping (OGM) retrospectively by analysing 11 samples from 10 cases with suspected or known TK rearrangements, previously analysed by current standard of care (SOC) methodologies, i.e., chromosome banding analysis (CBA), FISH and/or PCR‐based techniques. In six abnormal cases, OGM was able to detect the rearrangements previously determined by SOC methods. Furthermore, OGM identified the fusion partner in the JAK2 ‐ and PDGFRB ‐rearranged cases and elucidated the mechanism underlying the BCR::FGFR1 and ETV6::SYK rearrangement. In two cases with a normal karyotype, OGM detected two cryptic FIP1L1::PDGFRA and TNIP1::PDGFRB rearrangements. In the two remaining cases, no abnormalities were detected either by OGM or SOC methods. We demonstrate that OGM is a valid technique for the diagnostic workflow of MLN‐TK, able to detect TK rearrangements and to identify unknown TK fusion partners.