欧洲白血病网（ELN）最近提出了一项修订版的关于成人急性髓系白血病（AML）的诊断和管理建议，被称为ELN-2022。然而，在一个大的真实世界队列中的验证仍然缺乏。在本研究中，我们旨在在一个809例原发性、非M3类型，年轻（18-65岁）AML患者的队列中，验证ELN-2022的预后相关性。106例（13.1％）患者的风险类别从ELN-2017的决定重新分类为ELN-2022的决定。ELN-2022有效地帮助将患者区分为有利、中等和不利的风险组，评估其缓解率和生存率。在达到第一次完全缓解的患者中，异基因移植对中等风险组有益，但对有利或不利风险组则不利。我们通过将具有t（8；21）（q22;q22.1）/RUNX1::RUNX1T1，KITHigh、JAK2或FLT3-ITDHigh突变的AML患者重新分类为中等风险子集，将具有t（7；11）（p15;p15）/NUP98::HOXA9以及合并突变DNMT3A和FLT3-ITD的AML患者重新分类为不利风险子集，将具有复杂或单体重组的AML患者、inv（3）（q21.3q26.2）或t（3；3）（q21.3；q26.2）/GATA2、MECOM（EVI1）或TP53突变的AML患者重新分类为非常不利风险子集。修订后的ELN-2022系统有效地帮助将患者区分为有利、中等、不利和非常不利的风险组。总之，ELN-2022有助于将年轻、强化治疗的患者分为三组并具有独特的结果；建议修订ELN-2022以进一步改善AML患者的风险分层。新的预测模型需要经过前瞻性验证。本文受版权保护。保留所有权利。

The European LeukemiaNet (ELN) recently proposed a revised recommendation for the diagnosis and management of acute myeloid leukemia (AML) in adults, recognized as ELN-2022. However, validation in a large real-world cohort remains lacking. In this study, we aimed to validate the prognostic relevance of the ELN-2022 in a cohort of 809 de novo, non-M3, younger (ages 18-65 years) AML patients receiving standard chemotherapy. The risk categories of 106 (13.1%) patients were reclassified from that determined using ELN-2017 to that determined using ELN-2022. The ELN-2022 effectively helped distinguish patients as favorable, intermediate, and adverse risk groups in terms of remission rates and survival. Among patients who achieved first complete remission (CR1), allogeneic transplantation was beneficial for those in the intermediate risk group, but not for those in the favorable or adverse risk groups. We further refined the ELN-2022 system by re-categorizing AML patients with t(8;21)(q22;q22.1)/RUNX1::RUNX1T1 with KIThigh , JAK2 or FLT3-ITDhigh mutations into the intermediate risk subset, AML patients with t(7;11)(p15;p15)/NUP98::HOXA9 and AML patients with co-mutated DNMT3A and FLT3-ITD into the adverse risk subsets, and AML patients with complex or monosomal karyotypes, inv(3)(q21.3q26.2) or t(3 ;3)(q21.3 ;q26.2)/GATA2,MECOM(EVI1) or TP53 mutation into the very adverse risk subset. The refined ELN-2022 system performed effectively to distinguish patients as favorable, intermediate, adverse and very adverse risk groups. In conclusion, the ELN-2022 helped distinguish younger, intensively treated patients into three groups with distinct outcomes; the proposed refinement of ELN-2022 may further improve risk stratification among AML patients. Prospective validation of the new predictive model is necessary. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved.