A RETROSPECTIVE COHORT ANALYSIS OF CHILDREN AND ADOLESCENTS WITH
LYMPHOBLASTIC LYMPHOMA IN LATIN AMERICA.
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.