全身性肥大细胞增多症
米多司他林
医学
肿瘤
肥大细胞
髓系白血病
髓样
白血病
骨髓增生性肿瘤
免疫学
癌症研究
病理
骨髓纤维化
骨髓
作者
Tsewang Tashi,Michael W. Deininger
标识
DOI:10.1016/j.iac.2023.04.009
摘要
Advanced systemic mastocytosis (AdvSM) is a heterogeneous group of disorders characterized by neoplastic mast cell-related organ damage and frequently associated with a myeloid neoplasm. The 3 clinical entities that comprise AdvSM are aggressive SM (ASM), SM-associated hematologic neoplasm, and mast cell leukemia. A gain-of-function KIT D816 V mutation is the primary oncogenic driver found in about 90% of all patients with AdvSM. Midostaurin, an oral multikinase inhibitor with activity against KIT D816V, and avapritinib, an oral selective KIT D816V inhibitor are approved for AdvSM.
科研通智能强力驱动
Strongly Powered by AbleSci AI