Clinical Pathology and Cancer Diagnostics, Karolinska University Laboratory, Karolinska Institute, Stockholm, Sweden.
Institute of Pathology and Molecular Pathology, Helios University Clinic Wuppertal, University of Witten/Herdecke, Wuppertal, Germany.
Histopathology. 2023 Dec;83(6):829-849. doi: 10.1111/his.15021. Epub 2023 Aug 8.
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.
伴嗜酸性粒细胞和酪氨酸激酶(TK)基因融合的髓系/淋系肿瘤(M/LN-eo)是一组罕见的造血肿瘤,具有广泛的临床和形态学表现。儿科病例越来越多。重要的是,并非所有病例都表现为慢性髓系白血病,且并非总是存在嗜酸性粒细胞增多。此外,由于细胞遗传学上的隐匿性异常,标准细胞遗传学和分子方法可能不足以诊断 M/LN-eo。因此,建议采用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)进行额外评估,以鉴定特定的 TK 基因融合。在国际共识分类(ICC)和世界卫生组织第五版分类(WHO-HAEM5)中,最近将具有 JAK2 和 FLT3 重排以及 ETV6::ABL1 融合的 M/LN-eo 作为正式成员加入了这一类别。此外,还描述了其他涉及 TK 基因的较少见的明确遗传改变。本研究是对伴有 TK 基因融合的 M/LN-eo 的更新,重点介绍了新实体,这些实体通过 2022 年在佛罗伦萨举行的第 21 届欧洲血液病理学协会大会(EAHP-SH)框架内由欧洲骨髓工作组(EBMWG)组织的骨髓研讨会提交的病例得到说明。进行了文献复习,包括伴有 TK 基因融合的儿童 M/LN-eo 病例。