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Review
. 2024 May;484(5):853-857.
doi: 10.1007/s00428-023-03693-5. Epub 2023 Nov 20.

T cell phenotype and lack of eosinophilia are not uncommon in extramedullary myeloid/lymphoid neoplasms with ETV6::FLT3 fusion: a case report and review of the literature

Jérémy Schoelinck  1 , Julie Gervasoni  2 , Yann Guillermin  3 , Emmanuel Beillard  2 , Daniel Pissaloux  4 , Catherine Chassagne-Clement  2
Affiliations
Review

T cell phenotype and lack of eosinophilia are not uncommon in extramedullary myeloid/lymphoid neoplasms with ETV6::FLT3 fusion: a case report and review of the literature

Jérémy Schoelinck et al. Virchows Arch. 2024 May.

Abstract

In the 2022, WHO and ICC classifications, myeloid/lymphoid neoplasms with eosinophilia (M/LN-eo) and tyrosine kinase gene fusions represent rare hematologic malignancies driven by rearrangements of PDGFRA, PDGFRB, FGFR1, JAK2, FLT3, and ETV6::ABL1 fusion. Eosinophilia is the most constant finding, whereas the clinicopathological features are quite heterogeneous, presenting as Chronic eosinophilic leukemia (CEL) NOS, myelodysplastic/myeloproliferative neoplasm (MDS/MPN), MDS, MPN, systemic mastocytosis (SM), T or B cell acute lymphoblastic leukemia/lymphoblastic lymphoma (ALL/LBL), acute myeloid leukemia (AML), blastic phase of MPN, or mixed phenotype acute leukemia (MPAL). Extramedullary involvement at diagnosis or during progression is common. Here, we report a very unusual case of myeloid/lymphoid neoplasm with ETV6::FLT3 fusion with a nodal presentation without associated eosinophilia. Our case draws attention to diagnostic pitfalls in these rare entities.

Keywords: FLT3 gene; Myeloid and lymphoid neoplasms with eosinophilia; Peripheral T cell lymphoma.

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References

    1. Gerds AT, Gotlib J, Bose P et al (2020) Myeloid/lymphoid neoplasms with eosinophilia and TK fusion genes, Version 3.2021, NCCN clinical practice guidelines in oncology. J Natl Compr Cancer Netw 18:1248–1269. https://doi.org/10.6004/jnccn.2020.0042 - DOI
    1. Khoury JD, Solary E, Abla O et al (2022) The 5th edition of the World Health Organization classification of haematolymphoid tumours: myeloid and histiocytic/dendritic neoplasms. Leukemia 36:1703–1719. https://doi.org/10.1038/s41375-022-01613-1 - DOI - PubMed - PMC
    1. Tzankov A, Reichard KK, Hasserjian RP et al (2023) Updates on eosinophilic disorders. Virchows Arch 482:85–97. https://doi.org/10.1007/s00428-022-03402-8 - DOI - PubMed
    1. Tang G, Tam W, Short NJ et al (2021) Myeloid/lymphoid neoplasms with FLT3 rearrangement. Mod Pathol 34:1673–1685. https://doi.org/10.1038/s41379-021-00817-7 - DOI - PubMed
    1. Belhassan K, Saadalla A, Hoppman NL, et al. (2022) AMP case report: EV6/FLT3 fusion gene detected in a patient with T-cell lymphoblastic lymphoma.

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