Familial eosinophilia: a benign disorder?

Klion, Amy D.; Law, Melissa A.; Riemenschneider, William K.; McMaster, Mary L.; Brown, Margaret R.; Horne, McDonald K.; Karp, Barbara I.; Robinson, Michael R.; Sachdev, Vandana; Tucker, Eben; Turner, Maria L.; Nutman, Thomas B.

doi:10.1182/blood-2003-11-3850

Cited by 70 publications

(48 citation statements)

References 26 publications

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“…48 Of note, although 2 members of the family developed HES with fatal endomyocardial fibrosis and neuropathy, most affected family members have remained asymptomatic despite lifelong HE. 49 …”

Section: Familial He/hesmentioning

confidence: 99%

How I treat hypereosinophilic syndromes

Klion

2015

Blood

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199

126

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Hypereosinophilic syndromes (HESs) are a group of rare disorders characterized by peripheral blood eosinophilia of 1.5 × 109/L or higher and evidence of end organ manifestations attributable to the eosinophilia and not otherwise explained in the clinical setting. HESs are pleomorphic in clinical presentation and can be idiopathic or associated with a variety of underlying conditions, including allergic, rheumatologic, infectious, and neoplastic disorders. Moreover, the etiology of the eosinophilia in HESs can be primary (myeloid), secondary (lymphocyte-driven), or unknown. Although corticosteroids remain the first-line therapy for most forms of HESs, the availability of an increasing number of novel therapeutic agents, including tyrosine kinase inhibitors and monoclonal antibodies, has necessarily altered the approach to treatment of HESs. This review presents an updated treatment-based approach to the classification of patients with presumed HES and discusses the roles of conventional and novel agents in the management of these patients.

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Section: Familial He/hesmentioning

confidence: 99%

How I treat hypereosinophilic syndromes

Klion

2015

Blood

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199

126

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“…Early studies investigated eosinophilia as a potential biomarker [68][69][70][71][72]. One study showed variations in immune response within seemingly homogeneous IgE-mediated asthma phenotypes [73].…”

Section: Phenotypes To Asthma Endotypesmentioning

confidence: 99%

Characterizing wheeze phenotypes to identify endotypes of childhood asthma, and the implications for future management

Belgrave

Čustović

Simpson

2013

Expert Review of Clinical Immunology

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It is now a commonly held view that asthma is not a single disease, but rather a set of heterogeneous diseases sharing common symptoms. One of the major challenges to treating asthma is understanding these different asthma phenotypes and their underlying biological mechanisms.This review gives an epidemiological perspective of our current understanding of the different phenotypes that develop from birth to childhood that come under the umbrella term "asthma". The review focuses mainly on publications from longitudinal birth cohort studies where the natural history of asthma symptoms is observed over time in the whole population. Identifying distinct pathophysiological mechanisms for these different phenotypes will potentially elucidate different asthma endotypes, ultimately leading to more effective treatment and management strategies.

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“…Upon activation, eosinophils express a wide array of activation markers, such as CD23, CD25, HLA-DR, etc. (Klion et al 2004). Moreover, as mature lymphocytes, successful eosinophil activation also requires interaction mediated by co-stimulatory molecules by CD28/B7-1/B7-2 pathway , Shi 2004.…”

mentioning

confidence: 99%

High expression of co-stimulatory and adhesion molecules are observed on eosinophils during human Schistosoma mansoni infection

Silveira-Lemos

Teixeira-Carvalho

Martins‐Filho

et al. 2006

Mem. Inst. Oswaldo Cruz

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Herein we have focused attention on major phenotypic features of peripheral blood eosinophils from chronic Schistosoma mansoni-infected patients. For this purpose, detailed immunophenotypic profiles of a range of cell surface markers were performed, including activation markers (CD23/CD69/CD25/HLA-DR), co-stimulatory molecules (CD28/CD80/CD86), chemokine receptors (CXCR1/CXCR2/CCR3/CCR5) besides L-selectin-CD62L and adhesion molecules (CD18/CD54). Our major findings pointed out increased frequency of CD23 + -cells, besides decreased percentages of CD69 + -eosinophils, suggesting a chronic activation status with low frequency of early activated eosinophils in chronic S. mansoni-infected patients (INT) in comparison to non-infected individuals (NI). Moreover, a dichotomic expression of beta-chemokine receptors was observed during human schistosomiasis mansoni with higher CCR5 and lower levels of CCR3 observed between groups. Enhanced expression of co-stimulatory receptors (CD28/CD86) and adhesion molecules (CD54/CD18), besides striking lower frequency of L-selectin + were reported for eosinophils from INT group as compared to NI. Interestingly, the frequency of CD62L + -eosinophils and a range of cell activation related molecules pointed out an opposite pattern of association in NI and INT, where only INT patients that display lower frequency of CD62L + -eosinophils (first CD62L tertile) kept the unusual relationship between the expression of L-selectin and the CD23 activation marker. These findings suggest that distinct dynamic of activation markers expressed by eosinophils may occur during chronic S. mansoni infection.Key words: schistosomiasis -eosinophils -activation markers -L-selectin Eosinophils have been shown to play a major role in the immune response to helminthic infections (Butterworth et al. 1979, Hamann et al. 1987. In this context, transendothelial migration is essential for eosinophil recruitment from blood vessels into inflammatory tissues. At the inflammatory site, further interaction with invading parasites occurs via adherence with subsequent larval damage mediated by release of eosinophil toxic granular effector molecules, proinflammatory lipids, and cytokines (Greene et al. 1981, Medina-De la Garza et al. 1990, Strote et al. 1990, Brattig et al. 1991, Wildenburg et al. 1994. The prerequisite for adherence of effector eosinophils to parasite's larval stages is their sequential activation and chemotactic attraction that favor the eosinophils tethering. The migration of mature activated eosinophils from circulatory pools into the inflammatory foci depends on specific upregulation of adhesion molecules and chemokine receptors, which direct transmigration through a positive chemotactic gradient. This phenomenon seems to represent the key element for eosinophils recruitment that lead to accumulation of these proinflammatory cells that surround the parasite in its microenvironment (Pearlman 1997, Tachimoto et al. 2000.The profile of cell surface molecules expression on mature eosinophils resembles...

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Familial eosinophilia: a benign disorder?

Cited by 70 publications

References 26 publications

How I treat hypereosinophilic syndromes

How I treat hypereosinophilic syndromes

Characterizing wheeze phenotypes to identify endotypes of childhood asthma, and the implications for future management

High expression of co-stimulatory and adhesion molecules are observed on eosinophils during human Schistosoma mansoni infection

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