Progress in the treatment of adult acute myeloid leukaemia with NUP98 rearrangement
Acute myeloid leukaemia(AML)with NUP98 rearrangement is classified as a new separate cate-gory of AML with defining genetic abnormalities in the 5th edition of the World Health Organization Classification of Haematolymphoid Tumours.This rare subtype is usually relatively common in children with AML but only oc-curs in about 2％of adults.NUP98 gene can participate in the pathogenesis of AML with a variety of fusion part-ners,including HOX genes and non-HOX genes,and is often accompanied by other mutations such as FLT3-ITD,WT1,and NRAS.In adult AML,NUP98 gene rearrangements are mainly dominated by NUP98 ∷HOXA9 and NUP98 ∷ NSD1.Various experiments have demonstrated that the NUP98 fusion oncoproteins can drive leukemogenesis by affecting transcriptional regulation,chromatin remodeling,and other mechanisms.Pa-tients with NUP98 rearrangement exhibit distinctive clinical features,including evident bleeding symptoms and a worse outcome,with a higher prevalence among young females.Currently,the treatment of NUP98 rearrange-ment AML is confronted with challenges,lacking specific targeted drugs.Nevertheless,allogeneic hematopoietic stem cell transplantation has shown significant efficacy in improving prognosis.Therefore,NUP98-rearranged AML defined as a rare and high-risk leukemia subset,requires further more extensive research to develop better therapeutic strategies.
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