Millot Frédéric, Ampatzidou Mirella, Moulik Nirmalya Roy, Tewari Sanjay, Elhaddad Alaa, Hammad Mahmoud, Pichler Herbert, Lion Thomas, Tragiannidis Athanasios, Shima Haruko, An Wenbin, Yang Wenyu, Karow Axel, Farah Roula, Luesink Maaike, Dworzak Michael, Sembill Stephanie, De Moerloose Barbara, Sedlacek Petr, Schultz Kirk R, Kalwak Krzysztof, Versluys Birgitta, Athale Uma, Hijiya Nobuko, Metzler Markus, Suttorp Meinolf
Inserm CIC 1402, University Hospital of Poitiers, Poitiers, France.
Department of Pediatric Hematology Oncology (T.A.O.), Aghia Sophia Children's Hospital, Athens, Greece.
Leukemia. 2025 Apr;39(4):779-791. doi: 10.1038/s41375-025-02543-4. Epub 2025 Mar 5.
The treatment strategy for children and adolescents with chronic myeloid leukemia in the chronic phase (CML-CP) has evolved from allogeneic hematopoietic stem cell transplantation (HSCT) to tyrosine kinase inhibitors (TKIs). With the advent of next-generation TKIs and new targeted therapies in the CML field, an international pediatric CML expert panel provides recommendations based on the medical literature (including previous pediatric guidelines), national standards, and treatment principles used in adults with CML-CP. Recommendations include diagnosis of the disease and details on managing the initial steps of care of children and adolescents with newly diagnosed CML-CP, including complications such as leukostasis. The treatment recommendations are based on the initiation of therapy with a first- or second-generation TKI according to the allocated European Treatment and Outcome Study (EUTOS) long-term survival score risk group of the patient. The subsequent steps are based on the results of recommended monitoring which can justify a switch to another TKI or a drug in development if there is resistance or toxicity. The panel also provides recommendations regarding the discontinuation criteria for TKIs in children and adolescents in sustained deep molecular response. Allogeneic HSCT is not recommended as the first-line of treatment for children with CML-CP but is to be considered in case of progression to the advanced phase or failure of several lines of treatment. The present treatment and management recommendations are intended to provide advice to clinicians in view of optimizing the care and the outcome of children and adolescents with CML-CP.
慢性期儿童和青少年慢性髓性白血病(CML-CP)的治疗策略已从异基因造血干细胞移植(HSCT)演变为酪氨酸激酶抑制剂(TKIs)。随着新一代TKIs以及CML领域新靶向疗法的出现,一个国际儿科CML专家小组根据医学文献(包括既往儿科指南)、国家标准以及成人CML-CP的治疗原则给出了建议。建议内容包括疾病诊断以及新诊断CML-CP的儿童和青少年初始治疗步骤的详细信息,包括白细胞淤滞等并发症。治疗建议依据患者分配的欧洲治疗与预后研究(EUTOS)长期生存评分风险组,以第一代或第二代TKI开始治疗。后续步骤基于推荐监测的结果,如果出现耐药或毒性,这些结果可以证明需要换用另一种TKI或一种正在研发的药物。该小组还给出了处于持续深度分子反应的儿童和青少年停用TKIs的标准建议。异基因HSCT不建议作为CML-CP儿童的一线治疗,但在进展到晚期或多线治疗失败的情况下应予以考虑。目前的治疗和管理建议旨在为临床医生提供建议,以优化CML-CP儿童和青少年的治疗及预后。