医学
髓样
嗜酸性粒细胞增多症
肿瘤
索拉非尼
融合基因
溴尿嘧啶
淋巴瘤
嗜酸性粒细胞
肿瘤科
免疫学
内科学
病理
生物
基因
肝细胞癌
生物化学
哮喘
乙酰化
作者
Monica Cheng Munthe‐Kaas,Rakel Brendsdal Forthun,Atle Brendehaug,Anette K. Eek,Trude Høysæter,Liv Osnes,Trine Prescott,Signe Spetalen,Randi Hovland
标识
DOI:10.1097/mph.0000000000001890
摘要
Dysregulated tyrosine kinases in myeloid/lymphoid neoplasms with eosinophilia are rare, but do occur in children. To increase awareness of this diagnosis, we present a child who was diagnosed after a 3-year disease history. The patient was initially treated according to a T-cell lymphoblastic lymphoma protocol, but genetic analyses at recurrence revealed microdeletions resulting in an in-frame fusion of ZMYM2 and FLT3. Treatment with sorafenib, an FLT3 tyrosine kinase inhibitor, rapidly resulted in significant reduction of lymphadenopathy and normalization of white blood cell and eosinophil counts. At 17 months of treatment, he remains in complete hematologic, but not molecular remission.
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