Adult Acute Myeloid Leukemia with <i>NUP98</i> Rearrangement: An Underrecognized Entity, Particularly in East Asian Populations

edition of the WHO and the 2022 International Consensus Classification (ICC) guidelines, each indicating poor prognosis [2, 3].The WHO 5th edition classifies AML with NUP98 rearrangement as a distinct entity, with an incidence rate of approximately 2.3% in adults [4].The 2022 ICC categorizes it under AML with rare recurring translocations, specifically including NUP98::NSD1, NUP98::KMT5A, and other fusion partners [3].According to the ICC 2022 guidelines, these rearrangements are predominantly observed in infants and children.The WHO reports a pediatric incidence ranging from 2% to 4.8%, typically affecting patients aged 10 to 15 years.In contrast, data on adult cases remain limited.A review of previous cohorts shows that several Western studies report NUP98 rearrangement frequencies of 2.8% [4] and 2.5%[5], respectively.In contrast, more recent East Asia studies suggest a higher prevalence.For example, a Korean single-center study on adult AML detected NUP98 rearrangements in 6 of 119 patients (5.0%) using RNA-based sequencing [6].Similarly, a large NUP98, a component of the nuclear pore complex, functions as an aberrant transcription factor when involved in fusion proteins[1], with partner genes typically containing transcription-related domains.Acute myeloid leukemia (AML) with NUP98 rearrangement is recognized as a distinct diagnostic entity in both the 5th