A Retrospective Cohort Analysis of Children and Adolescents With Lymphoblastic Lymphoma in Latin America

IF 2.4 3区医学 Q2 HEMATOLOGY

Pediatric Blood & Cancer Pub Date : 2025-03-30 DOI:10.1002/pbc.31674

Magdalena Schelotto, Claudia Garrido, Jaqueline Montoya, Bruno Cuturi, Ana Rosa Braz, Flavio Luisi, Mario Alberto Ornelas Sanchez, Wendy Gomez, Pascale Gassant, Kenia Miller, Armando Peña, Godwin Job, Hilmarie Muniz-Talavera, Meenakshi Devidas, Victor M. Santana, Sima Jeha, Paola Friedrich, Monika L. Metzger, Guillermo L. Chantada

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Abstract

Background

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.

Methods

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.

Results

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).

Conclusions

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.

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拉丁美洲儿童和青少年淋巴母细胞淋巴瘤回顾性队列分析。

背景：关于低收入和中等收入国家儿童淋巴母细胞淋巴瘤（LLy）的信息很少。因此，在这里，我们描述了拉丁美洲一大批患有LLy的儿童和青少年的临床特征、治疗和结果。方法：回顾性分析St. Jude全球联盟成员的10家机构（2007-2017年）治疗的LLy患儿。结果：共纳入126例患者：t细胞LLy 60例（47.6%），前体b细胞LLy 49例（38.9%），免疫表型未知的LLy 17例（13.5%）。97例（77%）出现III/IV期疾病，42例（33.3%）诊断时有并发症。在30例（23.8%）病例中，病理诊断在活检后15天以上才得到，23例（18.2%）病例需要在其他机构进行病理复查。5年无事件生存率和总生存率分别为73%和78%。放弃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）、放弃治疗（n = 11）、诱导期间死亡（n = 4）、完全缓解期间死亡（n = 4）和第二次恶性肿瘤（n = 1）。结论：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风险疾病，许多患者病情危重，导致相对较高的毒性死亡率。在拉丁美洲，改善诊断、支持性措施和随访是减少治疗放弃和改善小儿LLy患者预后的必要条件。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric Blood & Cancer 医学-小儿科

CiteScore

4.90

自引率

9.40%

发文量

546

审稿时长

1.5 months

期刊介绍： Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.