Department of Pediatric Oncology, Hospital Manuel de Jesus Rivera La Mascota, Managua, Nicaragua.
National Pediatric Oncology Unit, Guatemala City, Guatemala.
Cancer. 2023 Mar 1;129(5):771-779. doi: 10.1002/cncr.34572. Epub 2022 Dec 12.
Children with relapsed acute lymphoblastic leukemia (ALL) in low-income and middle-income countries rarely survive. The Pediatric Hematology-Oncology Association of Central America (AHOPCA) developed the AHOPCA-ALL REC 2014 protocol to improve outcomes in resource-constrained settings without access to stem cell transplantation.
The AHOPCA-ALL REC 2014 protocol was based on a modified frontline induction phase 1A, a consolidation therapy with six modified R-blocks derived from the ALL-Berlin-Frankfurt-Munster REZ 2002 protocol and intermittent maintenance therapy. Children with B-lineage ALL were eligible after a late medullary relapse, an early or late combined relapse, or any extramedullary relapses. Those with T-lineage ALL were eligible after early and late extramedullary relapses, as were those with both B-lineage and T-lineage relapses occurring at least 3 months after therapy abandonment.
The study population included 190 patients with T-lineage (n = 3) and B-lineage (n = 187) ALL. Of those with B-lineage ALL, 25 patients had a very early extramedullary relapse, 40 had an early relapse (32 extramedullary and 8 combined), and 125 had a late relapse (34 extramedullary, 19 combined, and 72 medullary). The main cause of treatment failure was second relapse (52.1%). The 3-year event-free survival rate (± standard error) was 25.9% ± 3.5%, and the 3-year overall survival rate was 36.7% ± 3.8%. The 3-year event-free survival rate was 47.2% ± 4.7% for late relapses. The most frequently reported toxicity was grade 3 or 4 infection. Mortality during treatment occurred in 17 patients (8.9%), in most cases because of infectious complications.
Selected children with relapsed ALL in Central America can be cured with second-line regimens even without access to consolidation with stem cell transplantation. Children in low-income and middle-income countries who have lower risk relapses of ALL should be treated with curative intent.
在中低收入国家，复发的急性淋巴细胞白血病（ALL）患儿很少能够存活。中美洲儿科学血液学-肿瘤学协会（AHOPCA）制定了 AHOPCA-ALL REC 2014 方案，旨在为资源有限、无法进行干细胞移植的地区改善治疗效果。
AHOPCA-ALL REC 2014 方案基于改良的一线诱导期 1A 方案，强化治疗采用源自 ALL-Berlin-Frankfurt-Munster REZ 2002 方案的 6 个改良的 R 块，以及间歇性维持治疗。B 细胞系 ALL 患儿在髓外复发、早期或晚期联合复发或任何髓外复发后有资格入组；T 细胞系 ALL 患儿在早期和晚期髓外复发后有资格入组；在停止治疗至少 3 个月后发生 B 细胞系和 T 细胞系复发的患儿也有资格入组。
本研究人群包括 190 例 T 细胞系（n=3）和 B 细胞系（n=187）ALL 患儿。在 B 细胞系 ALL 患儿中，25 例患儿发生非常早期髓外复发，40 例患儿发生早期复发（32 例髓外复发和 8 例联合复发），125 例患儿发生晚期复发（34 例髓外复发、19 例联合复发和 72 例髓内复发）。治疗失败的主要原因是第二次复发（52.1%）。3 年无事件生存率（±标准误差）为 25.9%±3.5%，3 年总生存率为 36.7%±3.8%。晚期复发患儿的 3 年无事件生存率为 47.2%±4.7%。最常报告的毒性是 3 级或 4 级感染。17 例患儿在治疗期间死亡（8.9%），大多数是因为感染并发症。
中美洲复发 ALL 患儿可以使用二线方案治愈，即使无法进行巩固性干细胞移植。中低收入国家具有低复发风险的 ALL 患儿应进行治愈性治疗。
