Case Comprehensive Cancer Center/University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute.
Stanford Cancer Institute.
J Natl Compr Canc Netw. 2020 Sep;18(9):1248-1269. doi: 10.6004/jnccn.2020.0042.
Eosinophilic disorders and related syndromes represent a heterogeneous group of neoplastic and nonneoplastic conditions, characterized by more eosinophils in the peripheral blood, and may involve eosinophil-induced organ damage. In the WHO classification of myeloid and lymphoid neoplasms, eosinophilic disorders characterized by dysregulated tyrosine kinase (TK) fusion genes are recognized as a new category termed, myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB or FGFR1 or with PCM1-JAK2. In addition to these aforementioned TK fusion genes, rearrangements involving FLT3 and ABL1 genes have also been described. These new NCCN Guidelines include recommendations for the diagnosis, staging, and treatment of any one of the myeloid/lymphoid neoplasms with eosinophilia (MLN-Eo) and a TK fusion gene included in the 2017 WHO Classification, as well as MLN-Eo and a FLT3 or ABL1 rearrangement.
嗜酸性粒细胞疾病和相关综合征是一组异质性的肿瘤性和非肿瘤性疾病，其特征为外周血中嗜酸性粒细胞增多，并可能涉及嗜酸性粒细胞诱导的器官损伤。在世界卫生组织（WHO）髓系和淋巴组织肿瘤分类中，以酪氨酸激酶（TK）融合基因失调为特征的嗜酸性粒细胞疾病被认为是一种新的类别，称为伴有嗜酸性粒细胞增多和 PDGFRA、PDGFRB 或 FGFR1 重排或伴 PCM1-JAK2 重排的髓系/淋巴组织肿瘤。除了这些上述的 TK 融合基因外，还描述了涉及 FLT3 和 ABL1 基因的重排。这些新的 NCCN 指南包括对任何一种伴有 TK 融合基因的伴有嗜酸性粒细胞增多的髓系/淋巴组织肿瘤（MLN-Eo）和 2017 年 WHO 分类中包含的融合基因的诊断、分期和治疗的建议，以及伴有 FLT3 或 ABL1 重排的 MLN-Eo。
