A RETROSPECTIVE COHORT ANALYSIS OF CHILDREN AND ADOLESCENTS WITH LYMPHOBLASTIC LYMPHOMA IN LATIN AMERICA.
Magdalena Schelotto 1, Claudia Garrido2, Jaqueline Montoya 3, Bruno Cuturi1, Ana Rosa Braz 4, Flavio Luisi5, Mario Alberto Ornelas Sanchez 6, Wendy Gomez 7, Pascale Gassant 8, Kenia Miller 9, Armando Peña 10, Godwin Job 11 , Hilmarie Muniz-Talavera11, Meenakshi Devidas 11, Victor M. Santana 11, Sima Jeha 11, Paola Friedrich 11, Monica Metzger 11, Guillermo Chantada 1, 12.
1- Hospital Pereira Rossell. Fundación Pérez Scremini. Montevideo, Uruguay
2- Unidad Nacional de Oncología Pediátrica (UNOP).Guatemala, Guatemala
3- Instituto Nacional de Enfermedades Neoplásicas (INEN), Lima, Perú.
4- Hospital de Câncer Infantojuvenil de Barretos. São Paulo, Brasil
5- GRAACC/Instituto de Oncología Pediátrica/UNIFESP. São Paulo, Brasil
6- Hospital general de Tijuana. Tijuana, México.
7- Hospital infantil Robert Reid Cabral. Republica Dominicana.
8- Hospital Sant Damien. Haiti
9- Hospital del Niño Jose Renan Esquivel. Panamá
10- Hospital Escuela.Tegucigalpa, Honduras.
11- St. Jude Children’s Research Hospital. Memphis, United States of America.
12- Hospital Sant Joan de Déu, Barcelona, España.
Correspondence author: Magdalena Schelotto. Fundacion Perez Scremini. Hospital Pereira Rossell. Bulevar Artigas 1556. CP 11600 Montevideo Uruguay. Email: mgschelotto@gmail.com. +598 99281009
Running title: Lymphoblastic lymphoma in Latin America
Keywords: Lymphoblastic Lymphoma, non Hodgkin Lymphoma, children and adolescents, Latin America, St. Jude Global Alliance.
Word count: 2229
Abstract word count: 247
Number of Tables: 3
Number of Figures: 6
ABSTRACT
BACKGROUND AND AIMS:  There is scarce information about pediatric lymphoblastic lymphoma (LLy) in low and middle-income countries. We describe the clinical characteristics, treatment and outcome of a cohort of children and adolescents with LLy in Latin America (LA).
METHODS: Retrospective study analyzing pediatric patients with LLy in 10 institutions of the St. Jude Global Alliance from nine LA countries between 2007 and 2017.
RESULTS: One-hundred and twenty-six patients were included. Sixty (47.6%) had T-LLy, 49 (38.9%) B-LLy and in 17 (13.5%) the immunophenotype was unknown. Ninety-seven (77%) presented with stage III/IV disease, and 42 (33.3%) in critical conditions. In 30 cases (23.8%), the results of pathology diagnosis exceeded 15 days from biopsy, and 23 patients (18%) required a review at another institution.  The EFS and OS at 5 years were 73% (SE 0.047) and 78% (SE 0.0435), respectively. Five-year abandonment-sensitive EFS and OS were 65% (SE 0.0477) and 70% (SE 0.0459), respectively. Events included relapse/progression (n=22), refractory disease (n =1) abandonment (n=11), induction death (n=4), death in complete remission (n=4), and second malignancies (n=1).
CONCLUSIONS: A balanced proportion of LLy-T and B phenotypes was observed. Diagnosis was a challenge. Most of the patients presented with high-risk disease, and many in critical conditions. Toxic deaths and abandonment represented nearly half of the events. Improvements in diagnosis, supportive measures and follow up are imperative to improve the outcomes of pediatric LLy in Latin America.
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