费城样 (Ph-like) 或 BCR::ABL1 样急性淋巴细胞白血病 (ALL) 是 B 细胞前体 ALL (B-ALL) 的常见高危亚型，其特征是存在多种基因改变，难以诊断并集中在不同的激酶和细胞因子受体激活的基因表达谱上，类似于 BCR::ABL1 阳性 ALL 的命名法。激酶激活基因驱动因素的存在促使人们在临床前模型和临床环境中研究基于酪氨酸激酶抑制剂 (TKI) 的治疗的疗效。对传统化疗的反应不足、诱导失败率高和持续的可测量残留病 (MRD) 阳性进一步支持了这一点，与其他 B-ALL 亚型相比，这意味着生存率较低。因此，创新方法正在进行中，包括将 TKI 与一线治疗方案相结合以及早期引入免疫治疗策略（单克隆抗体、T 细胞接合剂、药物偶联物和 CAR-T 细胞）。目前建议对首次完全缓解的成人 BCR::ABL1 样 ALL 患者进行同种异体造血细胞移植 (HSCT)。然而，结合新疗法、更准确的诊断和更敏感的 MRD 评估可能会改变这些患者的风险分层和移植指征。版权所有 © 2024 Elsevier Inc. 保留所有权利。

Philadelphia-like (Ph-like) or BCR::ABL1-like acute lymphoblastic leukemia (ALL) is a common high-risk subtype of B-cell precursor ALL (B-ALL) characterized by a diverse range of genetic alterations that challenge diagnose and converge on distinct kinase and cytokine receptor-activated gene expression profiles, resembling those from BCR::ABL1-positive ALL from which its nomenclature. The presence of kinase-activating genetic drivers has prompted the investigation in preclinical models and clinical settings of the efficacy of tyrosine kinase inhibitor (TKI)-based treatments. This was further supported by an inadequate response to conventional chemotherapy, high rates of induction failure and persistent measurable residual disease (MRD) positivity, which translate in lower survival rates compared to other B-ALL subtypes. Therefore, innovative approaches are underway, including the integration of TKIs with frontline regimens and the early introduction of immunotherapy strategies (monoclonal antibodies, T-cell engagers, drug-conjugates, and CAR-T cells). Allogeneic hematopoietic cell transplantation (HSCT) is currently recommended for adult BCR::ABL1-like ALL patients in first complete remission. However, the incorporation of novel therapies, a more accurate diagnosis and a more sensitive MRD assessment may modify the risk stratification and the indication for transplant in these patients.Copyright © 2024 Elsevier Inc. All rights reserved.