Paediatric Haematology-Oncology Unit, Department of Paediatrics, Christian Medical College, Vellore, India.
Department of Transfusion Medicine and Immunohaematology, Christian Medical College, Vellore, India.
Pediatr Hematol Oncol. 2023;40(5):475-484. doi: 10.1080/08880018.2023.2209158. Epub 2023 Jul 21.
Managing a child with acute lymphoblastic leukemia (ALL) after relapse is arduous in low- and middle-income countries. A file review of children aged ≤15 years diagnosed with relapsed ALL from 2010 to 2019 was performed. Classification of relapse followed the Berlin-Frankfurt-Münster (BFM) scheme. The majority of patients were treated with a modified ALL-REZ-BFM protocol. Of 764 children treated for ALL in the study period, 163 (21.3%) relapsed. The median age at relapse was 101 months (range: 8-297). The immunophenotype was B-ALL and T-ALL in 140 (86%) and 23 (14%) patients. The site of relapse was extramedullary, combined, and medullary in 46 (28%), 45 (28%), and 72 (44%) patients. Very early, early, and late relapses were observed in 57 (35%), 66 (40%), and 40 (25%) patients. The proportions of extramedullary and medullary sites were greater among patients with early and late relapses, respectively ( = 0.039). Eighty-four (52%) patients were treated with palliative intent. The 2-year event-free survival (EFS) of patients treated with curative intent was 36.3 ± 6.3%. The 2-year EFS for very early/early and late relapses were 18.2 ± 6.2% and 67.6 ± 10.4% ( < 0.001). The 2-year EFS did not differ between extramedullary, combined, and medullary relapses. Treatment-related mortality occurred in 14 (20%) patients. More than 50% of the patients with relapse were treated with the intent of palliation. Extramedullary relapses were more likely to be early and did not have a better outcome than medullary relapses. Children with late relapse had a fair chance of survival with chemotherapy.
在中低收入国家，管理急性淋巴细胞白血病（ALL）复发患儿是一项艰巨的任务。对 2010 年至 2019 年期间诊断为复发 ALL 的≤15 岁儿童进行了病历回顾。根据柏林-法兰克福-明斯特（BFM）方案对复发进行分类。大多数患者接受了改良 ALL-REZ-BFM 方案治疗。在研究期间，764 名 ALL 患儿接受了治疗，其中 163 名（21.3%）复发。复发中位年龄为 101 个月（范围：8-297）。免疫表型为 B-ALL 和 T-ALL 的患儿分别为 140 例（86%）和 23 例（14%）。46 例（28%）、45 例（28%）和 72 例（44%）患儿的复发部位分别为髓外、髓内和髓外合并。57 例（35%）、66 例（40%）和 40 例（25%）患儿分别为极早、早和迟复发。极早期和早期复发患儿的髓外部位比例更高（ = 0.039）。84 例（52%）患儿姑息治疗。根治性治疗患儿的 2 年无事件生存率（EFS）为 36.3 ± 6.3%。极早期/早期和晚期复发患儿的 2 年 EFS 分别为 18.2 ± 6.2%和 67.6 ± 10.4%（ < 0.001）。髓外、髓内和髓外合并复发患儿的 2 年 EFS 无差异。14 例（20%）患儿发生治疗相关死亡。超过 50%的复发患儿接受姑息治疗。髓外复发更可能是早期的，但与髓内复发相比，结局并不更好。晚期复发患儿接受化疗仍有良好的生存机会。
