Hematology-Oncology Unit, Department of Pediatrics, Advanced Pediatrics Center, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Indian J Pediatr. 2020 Jun;87(6):443-450. doi: 10.1007/s12098-020-03234-x. Epub 2020 Feb 21.
Treatment with tyrosine kinase inhibitors (TKI) is an effective therapy for children and adolescents with chronic phase of chronic myeloid leukemia (CML). For the majority of patients with CML in low- and middle-income countries (LMIC), imatinib is the TKI of choice for reasons of cost, availability, and experience. Children are exposed to therapy for a greater proportion of their lifetime as compared to adults. The adverse effects of prolonged administration of TKI is a subject of ongoing research, as more experience is collected. Therapy with TKI is currently considered to be life-long. Trials on stopping treatment are ongoing to explore if it may be feasible in selected patients, as reported in adults. Growth-failure is a concerning adverse effect. Currently, it seems unclear if the final height attained is within the expected range of the mid-parenteral height and growth standards. Whether the children will achieve a normal height at the end of their growth potential or remain below the predicted range is critical to decide if therapeutic interventions (E.g., growth hormone therapy, or interruption in TKI) should be considered during the period of growth potential. Research on CML in children is at a relatively slow pace, largely due to the rarity of the disease. This provides a unique opportunity for research in population-dense LMICs, as several tertiary centers tend to have a sizable cohort of children and adolescents with CML on follow-up. This narrative summarises the challenges and opportunities in dealing with CML in children, particularly in reference to a center in LMIC.
酪氨酸激酶抑制剂(TKI)治疗是儿童和青少年慢性髓性白血病(CML)慢性期的有效治疗方法。对于中低收入国家(LMIC)的大多数 CML 患者,由于成本、可及性和经验原因,伊马替尼是 TKI 的首选。与成人相比,儿童在其一生中接受治疗的比例更高。由于积累了更多的经验,TKI 长期给药的不良反应是正在研究的课题。目前认为 TKI 治疗是终身的。正在进行停止治疗的试验,以探索在某些患者中是否可行,如在成人中报告的那样。生长障碍是一个令人担忧的不良反应。目前,似乎不清楚最终达到的身高是否在中亲身高和生长标准的预期范围内。儿童在生长潜力结束时是否能够达到正常身高,或者是否仍然低于预测范围,对于决定是否应在生长潜力期间考虑治疗干预(例如生长激素治疗或中断 TKI)至关重要。儿童 CML 的研究进展相对缓慢,主要是因为该疾病较为罕见。这为人口密集的中低收入国家的研究提供了一个独特的机会,因为几个三级中心往往有相当数量的儿童和青少年患有 CML 并在接受随访。本叙述总结了在处理儿童 CML 方面的挑战和机遇,特别是在中低收入国家的中心。