Clinical and Biological Markers in Hypereosinophilic Syndromes

Khoury, Paneez; Makiya, Michelle; Klion, Amy D.

doi:10.3389/fmed.2017.00240

Cited by 32 publications

(33 citation statements)

References 77 publications

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“…In the French series of 21 patients with L-HES, 7 out of 8 responded to IFN-α, making it the most effective commercialized treatment option in their experience ( 5 ). Another group reported a clear-cut reduction of absolute and relative CD3 − CD4 + T cell counts in response to IFN-α ( 30 ) similar to our observations herein. Such observations, together with the fact that one patient in our cohort was cured of disease with IFN-α, mitigates the clinical relevance of our previous in vitro investigations showing that IFN-α prolongs the survival of these cells in vitro in a cytokine-free milieu ( 31 ).…”

Section: Discussionsupporting

confidence: 90%

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

et al. 2020

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Section: Discussionsupporting

confidence: 90%

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

et al. 2020

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“…These biomarkers correlate with eosinophilic airway inflammation in asthma, but have not been explored more broadly in EADs. Additional examples of biomarkers with applicability restricted to a single or selected group of EADs include TARC/CCL17, or quantification of the clonal population in lymphoid HES, functional or structural markers of disease in EoE (e.g., EndoFLIP to measure esophageal distensibility), and tyrosine kinase mutations in myeloid HES. Further demonstration of the validity of these biomarkers is needed.…”

Section: Resultsmentioning

confidence: 99%

Revisiting the NIH Taskforce on the Research needs of Eosinophil-Associated Diseases (RE-TREAD)

Khoury

Akuthota

Ackerman

et al. 2018

Journal of Leukocyte Biology

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Eosinophil-associated diseases (EADs) are rare, heterogeneous disorders characterized by the presence of eosinophils in tissues and/or peripheral blood resulting in immunopathology. The heterogeneity of tissue involvement, lack of sufficient animal models, technical challenges in working with eosinophils, and lack of standardized histopathologic approaches have hampered progress in basic research. Additionally, clinical trials and drug development for rare EADs are limited by the lack of primary and surrogate endpoints, biomarkers, and validated patient-reported outcomes. Researchers with expertise in eosinophil biology and eosinophil-related diseases reviewed the state of current eosinophil research, resources, progress, and unmet needs in the field since the 2012 meeting of the NIH Taskforce on the Research of Eosinophil-Associated Diseases (TREAD). RE-TREAD focused on gaps in basic science, translational, and clinical research on eosinophils and eosinophil-related pathogenesis. Improved recapitulation of human eosinophil biology and pathogenesis in murine models was felt to be of importance. Characterization of eosinophil phenotypes, the role of eosinophil subsets in tissues, identification of biomarkers of eosinophil activation and tissue load, and a better understanding of the role of eosinophils in human disease were prioritized. Finally, an unmet need for tools for use in clinical trials was emphasized. Histopathologic scoring, patient- and clinician-reported outcomes, and appropriate coding were deemed of paramount importance for research collaborations, drug development, and approval by regulatory agencies. Further exploration of the eosinophil genome, epigenome, and proteome was also encouraged. Although progress has been made since 2012, unmet needs in eosinophil research remain a priority.

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“…Lymphocyte variant hypereosinophilic syndrome (L‐HES) is characterized by HE associated with circulating aberrant T cells, frequently with a Th2 cell immunophenotype CD3‐, CD4+, CD5bright+, and CD7−/partial+. These T cells are able to produce eosinophilopoietic cytokines such as IL‐4, IL‐5, and/or IL‐13, leading to overproduction of eosinophils .…”

Section: Discussionmentioning

confidence: 99%

A multimodality work‐up of patients with Hypereosinophilia

Boddu

Loghavi

et al. 2018

American J Hematol

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The work-up of patients with hypereosinophilia (HE) is complex. Following the recently revised World Health Organization criteria, we retrospectively reviewed 125 patients who were referred to us to exclude a neoplastic cause of HE (2003-2016). The clinical laboratory work-up confirmed secondary HE in 25 (20%) patients; myeloid/lymphoid neoplasms with rearrangements of PDGFRA (n = 9) or PDGFRB (n = 2) (9%); HE associated with a well-defined myeloid neoplasm in 8 (6%); and abnormal bone marrow and/or molecular genetic abnormalities consistent with chronic eosinophilic leukemia (CEL), not otherwise specified (NOS) in 21 (17%) patients. For the remaining 60 (48%) patients, a specific diagnosis was not identified, and 56 patients had HE related findings consistent with idiopathic hypereosinophilic syndrome (HES), while 4 patients who were asymptomatic. With a median follow up of 35.3 months (range, <1-104), patients with CEL, not otherwise specified (NOS) had a median OS of 26.1 months, significantly inferior to patients with idiopathic HES (not reached, P < .01). Thus, our experience in a single tertiary cancer center shows that the work-up of HE following WHO recommendations requires a multimodality-based approach; and a correct diagnosis determines risk stratification and proper patient management. However, the causes of HE remain unknown in approximately half of referred patients, indicating the need for further studies.

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Clinical and Biological Markers in Hypereosinophilic Syndromes

Cited by 32 publications

References 77 publications

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

Eosinophilia Associated With CD3−CD4+ T Cells: Characterization and Outcome of a Single-Center Cohort of 26 Patients

Revisiting the NIH Taskforce on the Research needs of Eosinophil-Associated Diseases (RE-TREAD)

A multimodality work‐up of patients with Hypereosinophilia

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