医学
全身性肥大细胞增多症
临床试验
重症监护医学
疾病
米多司他林
诊断试验
分类方案
内科学
儿科
白血病
数据科学
计算机科学
作者
Alessandro Nanni Costa,Emilia Scalzulli,Ida Carmosino,Marcello Capriata,Claudia Ielo,Chiara Masucci,Mauro Passucci,Maurizio Martelli,Massimo Breccia
标识
DOI:10.1080/14728214.2023.2221028
摘要
Systemic mastocytosis (SM) is a complex and heterogeneous disease, characterized by the clonal accumulation of mast cells in one or more organs. In 2022 both the World Health Organization (WHO) and the International Consensus Classification (ICC) modified the diagnostic and classification criteria of SM. Moreover, the identification of new clinical and molecular variables has improved prognostic tools and led to increasingly individualized therapeutic strategies.The aim of this review is to present the updates introduced by the International Consensus Classification in diagnostic criteria of SM. In addition, we report the latest data available from the most important clinical trials in patients both with non-advanced and advanced disease, including elenestinib and bezuclastinib.Diagnosis and classification of SM has evolved over years. The most recent WHO and ICC classification improved SM diagnostic work-up, providing clinicians with a clear and simplified diagnostic scheme. New approved targeted therapies such as midostaurin and avapritinib modified the treatment paradigm in patients in advanced stage, and next-generation inhibitors actually investigated in clinical trials are expected in the next future.
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