Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes

Valent, Peter; Klion, Amy D.; Roufosse, Florence; Simon, Dagmar; Metzgeroth, Georgia; Leiferman, Kristin M.; Schwaab, Juliana; Butterfield, Joseph H.; Sperr, Wolfgang R.; Sotlar, Karl; Vandenberghe, Peter; Hoermann, Gregor; Haferlach, Torsten; Moriggl, Richard; George, Tracy I.; Akin, Cem; Bochner, Bruce S.; Gotlib, Jason; Reiter, Andreas; Horny, Hans‐Peter; Arock, Michel; Simon, Hans‐Uwe; Gleich, Gerald J.

doi:10.1111/all.15544

Cited by 58 publications

(85 citation statements)

References 75 publications

(594 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The classification of eosinophilic disorders currently used for patient management and research is that proposed in 2011 by the International Cooperative Working Group on Eosinophil Disorders (ICOG-EO) which has recently been revised. Above all, this classification distinguishes blood eosinophilia (between 0.5 and 1.5 × 10 9 /L eosinophils), HE (blood eosinophilia > 1.5 × 10 9 /L and/or tissue eosinophilia) and HES [ 1 , 2 ]. Detailed criteria for each condition are listed in Box 1 .…”

Section: General Informationmentioning

confidence: 99%

“…HE of undetermined significance (HE US ) is defined as a condition in which HE is asymptomatic and idiopathic in nature [ 1 , 2 , 12 ]. There is no consensus definition in the literature, but experience in the field suggests that this diagnosis is based on the workup detailed in Box 4 (including lymphocyte phenotyping to search for cell populations of interest in the case of lymphocytic HE/HES, and FIP1L1::PDGFRA testing only in the presence of suggestive clinical or laboratory findings).…”

Section: Initial Assessmentmentioning

confidence: 99%

See 1 more Smart Citation

French guidelines for the etiological workup of eosinophilia and the management of hypereosinophilic syndromes

Groh

Rohmer

Etienne

et al. 2023

Orphanet J Rare Dis

View full text Add to dashboard Cite

Eosinophilic-related clinical manifestations are protean and the underlying conditions underpinning eosinophilia are highly diverse. The etiological workup of unexplained eosinophilia/hypereosinophilia can be challenging, and can lead sometimes to extensive, inappropriate, costly and/or invasive investigations. To date, guidelines for the etiological workup and management of eosinophilia are mainly issued by hematologists, and thus mostly cover the scope of clonal hypereosinophilic syndromes (HES). Here, thanks to an extensive literature review, and thanks to the joint work of a large panel of experts involving physicians from both adult and pediatric medicine and from various subspecialties (as well as a representative of a patients’ association representative), we provide recommendations for both the step-by step diagnostic workup of eosinophilia (whether unexplained or within specific contexts) as well as the management and follow-up of the full spectrum of eosinophilic disorders (including clonal, reactive, lymphocytic and idiopathic HES, as well as single-organ diseases). Didactic prescription summaries intended to facilitate the prescription of eosinophil-targeted drugs are also provided, as are practical diagnostic and therapeutic algorithms. Lastly, this set of recommendations also includes a summary intended for general practitioners, as well as an overview of the therapeutic patient education program set up by the French reference center for HES. Further updates will be mandatory as new validated information emerges.

show abstract

Section: General Informationmentioning

confidence: 99%

Section: Initial Assessmentmentioning

confidence: 99%

French guidelines for the etiological workup of eosinophilia and the management of hypereosinophilic syndromes

Groh

Rohmer

Etienne

et al. 2023

Orphanet J Rare Dis

View full text Add to dashboard Cite

show abstract

“…HES is considered as idiopathic (iHES) when an underlying primary cause (either reactive or neoplastic) cannot be identified. 2,82 HES is generally divided into 1 : (1) secondary HES (eosinophils are reactive and non-clonal) including lymphocyte variant-HES; (2) primary HES (associated with a hematopoietic neoplasms); (3) idiopathic HES.…”

Section: Idiopathic Hypereosinophilic Syndromesmentioning

confidence: 99%

The international consensus classification of eosinophilic disorders and systemic mastocytosis

et al. 2023

Self Cite

View full text Add to dashboard Cite

Based on new data and increased understanding of disease molecular genetics, the international consensus classification (ICC) has made several changes in the diagnosis and classification of eosinophilic disorders and systemic mastocytosis. Myeloid/lymphoid neoplasms with eosinophilia (M/LN‐eo) and gene rearrangements have been renamed as M/LN‐eo with tyrosine kinase gene fusions (M/LN‐eo‐TK). The category has been expanded to include ETV6::ABL1 and FLT3 fusions, and to accept PCM1::JAK2 and its genetic variants as formal members. The overlaps and differences between M/LN‐eo‐TK and BCR::ABL1‐like B‐lymphoblastic leukemia (ALL)/de novo T‐ALL sharing the same genetic lesions are addressed. Besides genetics, ICC for the first time has introduced bone marrow morphologic criteria in distinguishing idiopathic hypereosinophilia/hypereosinophilic syndrome from chronic eosinophilic leukemia, not otherwise specified. The major diagnostic criteria for systemic mastocytosis (SM) in the ICC remain largely based on morphology, but several minor modifications/refinements have been made in criteria related to diagnosis, subclassification, and assessment of disease burden (B‐ and C‐findings). This review is to focus on the ICC updates related to these disease entities, illustrated through changes related to morphology, molecular genetics, clinical features, prognosis, and treatment. Two practical algorithms are provided in navigating through the diagnosis and classification systems of hypereosinophilia and SM.

show abstract

“…374 Moreover, it requires the exclusion of any underlying disease or condition that might trigger HE, in addition to ruling out HES. 374…”

Section: | Une Xpl Ained Eos Inophiliamentioning

confidence: 99%

Eosinophils from A to Z

Gigon

Fettrelet

Yousefi

et al. 2023

Allergy

Self Cite

View full text Add to dashboard Cite

Eosinophils are bone marrow‐derived granulocytes and are found in low numbers in the peripheral blood of healthy subjects. In type 2 inflammatory diseases, eosinopoiesis in the bone marrow is increased, resulting in a rise in the number of mature eosinophils released in the circulation. From the blood, eosinophils can migrate in multiple tissues and organs under both physiological and pathological conditions. Eosinophils exert their various functions through the synthesis and release of a variety of granule proteins and pro‐inflammatory mediators. Despite being present in all species of vertebrates, the functional role of eosinophils is still a matter of debate. Eosinophils may play a role in host defense against various pathogens. In addition, eosinophils have been reported to be involved in tissue homeostasis and exhibit immunomodulatory activities. In this review, we aim to provide a broad overview of eosinophil biology and eosinophilic diseases in a lexicon‐style format using keywords starting from A until Z with cross‐references to other chapters indicated in italics in the text or specified in parentheses.

show abstract

Proposed refined diagnostic criteria and classification of eosinophil disorders and related syndromes

Cited by 58 publications

References 75 publications

French guidelines for the etiological workup of eosinophilia and the management of hypereosinophilic syndromes

French guidelines for the etiological workup of eosinophilia and the management of hypereosinophilic syndromes

The international consensus classification of eosinophilic disorders and systemic mastocytosis

Eosinophils from A to Z

Contact Info

Product

Resources

About