费城染色体阳性（Ph +）急性淋巴细胞淋巴瘤（ALL）是儿童中罕见的ALL亚型，在2-5％的病例中可见。诊断评估包括常规核型分析以及应用荧光原位杂交（FISH）或逆转录聚合酶链式反应（RT-PCR）检测BCR-ABL1易位。对于儿童，前线治疗包括密集化疗与伊马替尼（300-340mg/m2/d）或达沙替尼（60-80mg/m2/d）的联合。一旦获得BCR-ABL的结果，应在诱导期间立即引入伊马替尼/达沙替尼。最低残留病（MRD）监测是必不可少的；多参数流式细胞术和免疫球蛋白/ T细胞受体重排PCR是首选方法。脑脊液疗法至少包括12剂甲氨蝶呤用于中枢神经系统（CNS）预防，但对于CNS3累及，需要进行颅脑放射。鉴于MRD持续阳性/诱导失败的高风险病例，可能需要考虑在第一缓解阶段进行异基因造血干细胞移植（HSCT）。儿童的靶向治疗酪氨酸激酶抑制剂（TKI）的维持治疗颇有争议，存在潜在的长期不良影响。在复发时，TKI的选择受到BCR-ABL酪氨酸激酶结构域耐药突变的指导，尽管儿童中耐药突变的频率较低。如果未完成，异基因HSCT对于第二缓解的巩固至关重要。Ph样ALL是一种新认识的分子实体，其基因表达谱类似于Ph + ALL且存活结果差劲。在资源有限的环境中，应考虑对高风险患者进行分阶段的具有成本效益的诊断评估，以排除常见的遗传异常。目前的治疗策略与Ph阴性ALL相似。鼓励这类儿童参与临床试验，以评估潜在的靶向药物对这个亚型的疗效。© 2023. 作者（作者），在Dr. K C Chaudhuri基金会独家授权下。

Philadelphia chromosome positive (Ph+) acute lymphoblastic lymphoma (ALL) is an uncommon subtype of ALL in children, seen in 2-5% cases. Diagnostic evaluation includes conventional karyotyping and detection of BCR-ABL1 translocation by fluorescence in-situ hybridization (FISH) or reverse transcriptase polymerase chain reaction (RT-PCR). For children, the frontline management includes combination of intensive chemotherapy along with imatinib (300-340 mg/m2/d) or dasatinib (60-80 mg/m2/d). Imatinib/dasatinib should be introduced in induction as soon as results for BCR-ABL are available. Minimal residual disease (MRD) monitoring is essential; multi-parametric flowcytometry and immunoglobulin/T-cell receptor rearrangement PCR are the preferred methods. Intrathecal therapy with at least 12 doses of methotrexate is adequate for central nervous system (CNS) prophylaxis, but cranial radiation is necessary for CNS3 involvement. Allogeneic hematopoietic stem cell transplantation (HSCT) in first remission may be considered in high-risk cases (persistent MRD positivity/induction failure). Maintenance therapy with tyrosine kinase inhibitors (TKI) in children is debatable, with potential concerns for long term adverse effects. At relapse, the choice of TKI is guided by the presence of BCR-ABL tyrosine kinase domain resistance mutations, although the frequency of resistance mutations in children are lower. Allogeneic HSCT is essential for consolidation in second remission, if not done. Ph-like ALL is a newly recognized molecular entity, with gene expression profile similar to Ph+ALL and poor survival outcomes. In resource-constrained settings, a stepwise cost-effective diagnostic evaluation should be considered among high-risk patients without recurrent genetic abnormalities. Current treatment strategies remain similar to Ph-negative ALL. Enrolment in clinical trials is encouraged for such children to evaluate potential targeted agents in this subtype.© 2023. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.