Updates on eosinophilic disorders

Tzankov, Alexandar; Reichard, Kaaren K.; Arber, Daniel A.; Orazi, Attilio; Wang, Sa A.

doi:10.1007/s00428-022-03402-8

Cited by 23 publications

(29 citation statements)

References 75 publications

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“…Based on the classification proposed by the ICOG‐EO, eosinophilic leukemias can be divided into chronic eosinophilic leukemia (CEL) and acute eosinophilic leukemia (AEL) (Table S5). 15,23 The WHO and ICC classifications include CEL among the classical MPN, but do not include AEL 11,64 . Per the ICOG‐EO proposal, CEL is diagnosed when the percentage of (clonal) eosinophils in the PB and/or BM is ≥30% and the percentage of myeloblasts is <20% 15 .…”

Section: Neoplastic He (Hen) = Clonal Hementioning

confidence: 99%

“…The clinical impact of AEL remains uncertain given the rarity of this disease and the fact that the blast cell compartment (AML) is clinically more relevant than the impact of HE or HE‐induced organ dysfunction in patients with AEL. As mentioned, the current WHO and ICC classifications do not include AEL 11,64 …”

Section: Neoplastic He (Hen) = Clonal Hementioning

confidence: 99%

“…[8][9][10][11][15][16][17][18][19][20][21] In other patients, mutations in JAK1 or JAK2, or other key signal-transduction molecules, such as STAT5, are found. [61][62][63] The 2022-updated WHO classification and a recently proposed International Consensus Classification (ICC) 64 include additional kinase fusion genes associated with HE in a newly named diagnostic category: "myeloid/lymphoid neoplasms with eosinophilia and tyrosine kinase gene fusions (MLN-TK)" (Table S5). 11 Standard evaluations to screen for such fusion genes include S5 and Figure S1.…”

Section: Neopl a S Ti C He (He N ) = Clonal Hementioning

confidence: 99%

“…Based on these considerations, our faculty is of the opinion that the WHO and ICC classification of hematopoietic (stem cell and myeloid) neoplasms accompanied by HE should be followed, 11 , 64 but that the additional classification principles provided by the ICOG‐EO should also be applied. 15 , 23 Specifically, after the WHO‐related genetic markers are examined and the HE‐related primary pathology is documented (Figure 2 ), the presence or absence of HES should be addressed and the final diagnosis established according to a combination of WHO (ICC) and ICOG‐EO criteria (Figure 2 ).…”

Section: Neoplastic He ( He ...mentioning

confidence: 99%

“… 15 , 23 The WHO and ICC classifications include CEL among the classical MPN, but do not include AEL. 11 , 64 Per the ICOG‐EO proposal, CEL is diagnosed when the percentage of (clonal) eosinophils in the PB and/or BM is ≥30% and the percentage of myeloblasts is <20%. 15 Certain driver‐related myeloid‐, stem cell‐ and mast cell neoplasms (including CML and JAK2 V617F+ MPN) must be excluded as primary trigger of HE before a diagnosis of CEL can be established, unless co‐existence of such a neoplasm with CEL is demonstrated with certainty based on detailed histopathological and molecular studies (Table S5 ).…”

Section: Neoplastic He ( He ...mentioning

confidence: 99%

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

Valent

Klion

Roufosse

et al. 2022

Allergy

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Eosinophilia and eosinophil activation are recurrent features in various reactive states and certain hematologic malignancies. In patients with hypereosinophilia (HE), HE-induced organ damage is often encountered and may lead to the diagnosis of a hypereosinophilic syndrome (HES). A number of known mechanisms and etiologies contribute to the development of HE and HES. Based on these etiologies and the origin of eosinophils, HE and HES are divided into primary forms where eosinophils are clonal cells, reactive forms where an underlying reactive or neoplastic condition is detected and eosinophils are considered to be "non-clonal" cells, and idiopathic HE and HES in which neither a clonal nor a reactive underlying pathology is detected.Since 2012, this classification and the related criteria have been widely accepted and regarded as standard. However, during the past few years, new developments in the field and an increasing number of markers and targets have created a need to update these criteria and the classification of HE and HES. To address this challenge, a Working Conference on eosinophil disorders was organized in 2021. In this conference, a panel of experts representing the relevant fields, including allergy, dermatology, hematology, immunology, laboratory medicine, and pathology, met and discussed new markers and concepts as well as refinements in definitions, criteria and classifications of HE and HES. The outcomes of this conference are presented in this article and should assist in the diagnosis and management of patients with HE and HES in daily practice and in the preparation and conduct of clinical trials.

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Section: Neoplastic He (Hen) = Clonal Hementioning

confidence: 99%

Section: Neoplastic He (Hen) = Clonal Hementioning

confidence: 99%

Section: Neopl a S Ti C He (He N ) = Clonal Hementioning

confidence: 99%

Section: Neoplastic He ( He ...mentioning

confidence: 99%

Section: Neoplastic He ( He ...mentioning

confidence: 99%

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

Valent

Klion

Roufosse

et al. 2022

Allergy

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The international consensus classification of eosinophilic disorders and systemic mastocytosis

et al. 2023

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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.

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Concomitant myeloproliferative neoplasm with eosinophilia, B and T cell lymphoblastic lymphoma/leukemia and mast cell proliferation driven by ZMYM2::FGFR1 rearrangement

et al. 2023

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Updates on eosinophilic disorders

Cited by 23 publications

References 75 publications

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

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

The international consensus classification of eosinophilic disorders and systemic mastocytosis

Concomitant myeloproliferative neoplasm with eosinophilia, B and T cell lymphoblastic lymphoma/leukemia and mast cell proliferation driven by ZMYM2::FGFR1 rearrangement

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