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Updates on eosinophilic disorders

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Abstract

This review addresses changes and updates in eosinophilic disorders under the International Consensus Classification (ICC). The previous category of myeloid/lymphoid neoplasm with eosinophilia (M/LN-eo) and a specific gene rearrangement is changed to M/LN-eo with tyrosine kinase gene fusions to reflect the underlying genetic lesions. Two new members, M/LN-eo with ETV6::ABL1 fusion and M/LN-eo with various FLT3 fusions, have been added to the category; and M/LN-eo with PCM1::JAK2 and its genetic variants ETV6::JAK2 and BCR::JAK2 are recognized as a formal entity from their former provisional status. The updated understanding of the clinical and molecular genetic features of PDGFRA, PDGFRB and FGFR1 neoplasms is summarized. Clear guidance as to how to distinguish these fusion gene–associated disorders from the overlapping entities of Ph-like B-acute lymphoblastic leukemia (ALL), de novo T-ALL, and systemic mastocytosis is provided. Bone marrow morphology now constitutes one of the diagnostic criteria of chronic eosinophilic leukemia, NOS (CEL, NOS), and idiopathic hypereosinophilia/hypereosinophilic syndrome (HE/HES), facilitating the separation of a true myeloid neoplasm with characteristic eosinophilic proliferation from those of unknown etiology and not attributable to a myeloid neoplasm.

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Authors and Affiliations

  1. Institute of Medical Genetics and Pathology, University Hospital Basel, Basel, Switzerland

    Alexandar Tzankov

  2. Department of Laboratory Medicine and Pathology, Mayo Clinic, NY, Rochester, USA

    Kaaren K. Reichard

  3. Department of Pathology, Massachusetts General Hospital, MA, Boston, USA

    Robert P. Hasserjian

  4. Department of Pathology, University of Chicago, IL, Chicago, USA

    Daniel A. Arber

  5. Department of Pathology, Texas Tech University Health Sciences Center, El Paso, TX, USA

    Attilio Orazi

  6. Department of Hematopathology, Division of Pathology, The University of Texas MD Anderson Cancer Center, TX, Houston, USA

    Sa A. Wang

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  1. Alexandar Tzankov
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  2. Kaaren K. Reichard
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  3. Robert P. Hasserjian
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Contributions

AZ, RH, DA, AO, and SW participated in the discussion and formulation of the International Consensus Classification (ICC) on eosinophilic disorder. All authors participated in the writing, editing, and proofreading of the manuscript.

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Correspondence to Sa A. Wang.

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Tzankov, A., Reichard, K.K., Hasserjian, R.P. et al. Updates on eosinophilic disorders. Virchows Arch 482, 85–97 (2023). https://doi.org/10.1007/s00428-022-03402-8

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