Schelotto Magdalena, Garrido Claudia, Montoya Jaqueline, Cuturi Bruno, Braz Ana Rosa, Luisi Flavio, Sanchez Mario Alberto Ornelas, Gomez Wendy, Gassant Pascale, Miller Kenia, Peña Armando, Job Godwin, Muniz-Talavera Hilmarie, Devidas Meenakshi, Santana Victor M, Jeha Sima, Friedrich Paola, Metzger Monika L, Chantada Guillermo L
Hospital Pereira Rossell, Fundación Pérez Scremini, Montevideo, Uruguay.
Unidad Nacional de Oncología Pediátrica, Guatemala City, Guatemala.
Pediatr Blood Cancer. 2025 Jun;72(6):e31674. doi: 10.1002/pbc.31674. Epub 2025 Mar 30.
Information about pediatric lymphoblastic lymphoma (LLy) in low- and middle-income countries is scarce. Thus, here we describe the clinical characteristics, treatment, and outcome of a large cohort of children and adolescents with LLy in Latin America.
A retrospective analysis was conducted of pediatric patients with LLy treated at 10 institutions (2007-2017) that are members of the St. Jude Global Alliance.
A total of 126 patients were included: 60 (47.6%) had T-cell LLy, 49 (38.9%) had precursor B-cell LLy, and 17 (13.5%) had LLy with an unknown immunophenotype. Ninety-seven (77%) presented with stage III/IV disease, and 42 (33.3%) had complications at diagnosis. In 30 (23.8%) cases, the pathology diagnosis was received more than 15 days after the biopsy, and in 23 (18.2%) cases, a pathology review at another institution was required. The 5-year event-free survival and overall survival were 73% and 78%, respectively. Abandonment-sensitive event-free survival and overall survival were 65% and 70%, respectively. Events included disease relapse/progression (n = 22), refractory disease (n = 1), treatment abandonment (n = 11), death during induction (n = 4), death during complete remission (n = 4), and second malignancy (n = 1).
Diagnosis of pediatric LLy in Latin America was challenging; however, the proportions of T-cell and precursor B-cell immunophenotypes were balanced. Most patients presented with high-risk disease, and many had critical conditions, leading to a relatively high toxic death rate. Improvements in diagnosis, supportive measures, and follow-up are imperative to decrease treatment abandonment and improve the outcomes of pediatric patients with LLy in Latin America.
低收入和中等收入国家关于儿童淋巴细胞白血病（LLy）的信息匮乏。因此，我们在此描述拉丁美洲一大群儿童和青少年LLy患者的临床特征、治疗情况及预后。
对圣犹大全球联盟成员的10家机构（2007 - 2017年）治疗的儿童LLy患者进行回顾性分析。
共纳入126例患者：60例（47.6%）为T细胞LLy，49例（38.9%）为前体B细胞LLy，17例（13.5%）免疫表型未知。97例（77%）表现为III/IV期疾病，42例（33.3%）在诊断时有并发症。30例（23.8%）病例在活检后15天以上才获得病理诊断，23例（18.2%）病例需要在另一家机构进行病理复查。5年无事件生存率和总生存率分别为73%和78%。对放弃治疗敏感的无事件生存率和总生存率分别为65%和70%。事件包括疾病复发/进展（n = 22）、难治性疾病（n = 1）、治疗放弃（n = 11）、诱导期死亡（n = 4）、完全缓解期死亡（n = 4）和第二恶性肿瘤（n = 1）。
拉丁美洲儿童LLy的诊断具有挑战性；然而，T细胞和前体B细胞免疫表型的比例是平衡的。大多数患者表现为高危疾病，许多患者病情危急，导致毒性死亡率相对较高。必须改进诊断、支持措施和随访，以减少治疗放弃并改善拉丁美洲儿童LLy患者的预后。
