伴有嗜酸性白血病（M/LN-eo）和酪氨酸激酶（TK）基因融合的髓系/淋巴系肿瘤是一组罕见的造血系统肿瘤，临床和形态学表现广泛。越来越多的儿科病例被识别出来。重要的是，并非所有病例都表现为慢性髓细胞肿瘤，也并非都存在嗜酸性白血病。此外，标准的细胞遗传学和分子方法可能无法足够地诊断M/LN-eo，并且存在细胞遗传学上的隐性异常。因此，建议通过荧光原位杂交和其他分子遗传学技术（基于阵列的比较基因组杂交、RNA测序）进行进一步评估，以确定特定的TK基因融合。在国际共识分类（ICC）和第5版世界卫生组织分类（WHO-HAEM5）中，最近将具有JAK2和FLT3重排以及ETV6::ABL1融合的M/LN-eo正式纳入该类别。此外，还描述了涉及TK基因的其他较少见的确定性遗传改变。本研究是关于带有TK基因融合的M/LN-eo的更新，重点是通过提交给欧洲骨髓工作组（EBMWG）在第21届欧洲血液病理学协会（EAHP-SH）举办的骨髓研讨会中提供的病例来说明。进行了包括儿科M/LN-eo带TK基因融合的文献综述。© 2023年作者。由约翰威利父子有限公司出版的组织病理学。

Myeloid/lymphoid neoplasms with eosinophilia (M/LN-eo) and tyrosine kinase (TK) gene fusions are a rare group of haematopoietic neoplasms with a broad range of clinical and morphological presentations. Paediatric cases have increasingly been recognised. Importantly, not all appear as a chronic myeloid neoplasm and eosinophilia is not always present. In addition, standard cytogenetic and molecular methods may not be sufficient to diagnose M/LN-eo due to cytogenetically cryptic aberrations. Therefore, additional evaluation with fluorescence in-situ hybridisation and other molecular genetic techniques (array-based comparative genomic hybridisation, RNA sequencing) are recommended for the identification of specific TK gene fusions. M/LN-eo with JAK2 and FLT3-rearrangements and ETV6::ABL1 fusion were recently added as a formal member to this category in the International Consensus Classification (ICC) and the 5th edition of the WHO classification (WHO-HAEM5). In addition, other less common defined genetic alterations involving TK genes have been described. This study is an update on M/LN-eo with TK gene fusions with focus on novel entities, as illustrated by cases submitted to the Bone Marrow Workshop, organised by the European Bone Marrow Working Group (EBMWG) within the frame of the 21st European Association for Haematopathology congress (EAHP-SH) in Florence 2022. A literature review was performed including paediatric cases of M/LN-eo with TK gene fusions.© 2023 The Authors. Histopathology published by John Wiley & Sons Ltd.