30 Years of Experience with Non-Hodgkin Lymphoma in Children and Adolescents: a retrospective cohort study

Abstract

SUMMARY OBJECTIVE Describe the clinical and demographic characteristics of pediatric patients with non-Hodgkin’s lymphoma (NHL) enrolled in a tertiary unit of Pediatric Hematology between 1982-2015. PATIENTS AND METHODS A retrospective cohort study of 140 patients aged 16 years or less with NHL. Demographic characteristics, data on diagnosis, and outcomes were analyzed. The overall survival (OS) analysis and stratification by the most frequent histological subtypes were performed using the Kaplan-Meier method. RESULTS One hundred and thirty-six patients with de novo NHL and four with NHL as a second malignancy were analyzed. The median age at diagnosis was 6.4 years (interquartile range, 4.2 to 11.1 years); 101 patients were males. Four patients had primary immunodeficiency, four had human immunodeficiency virus, two post-liver transplantation, and one had autoimmune lymphoproliferative syndrome. The most frequent histological type was NHL of mature B- cell (B-NHL-B; 67.1%), with Burkitt’s lymphoma being the most frequent subtype, and lymphoblastic lymphoma (LBL, 21.4%). The main clinical manifestation at the diagnosis was abdominal tumors (41.4%). During the follow-up time, 13 patients relapsed, but five of them reached a second remission. Thirty-five patients died, and 103 remained alive in clinical remission. No contact was possible for two patients. The OS at 5 years was 74.5% (± 3.8%). The OS estimated for patients with LBL, NHL-B, and the remaining was 80.4%±7.9%, 72.8%±4.7%, and 74.5%±11%, respectively (P = 0.58). CONCLUSION Our results are comparable with cohorts from other middle-income countries.

Non Hodgkin Lymphoma Among Children: Pathological Aspects and Diagnostic Challenges

“…The median age at diagnosis in our study was 7.4 years (ranging from 5 months to 15 years). Our findings are older to the previously reported age in the literature 4 - 7 which ranges from (6.1 to 6.4 years). NHL in our study affected (64.3%) boys and (35.7%) girls with male to female ratio of 1.8; 1.these results are consistent with previously reported male predominance of childhood lymphoma in different series of the literature.…”

“… 1 , 3 Similar to different series of the literature, BL was the most common subtype in our patients, followed by LBL, DLBL and ALBL. 4 - 7 Multiple studies are required for the diagnosis of pediatric NHL and for determining prognostic factors; including: morphologic findings (cytology and histology); immunophenotyping (flow cytometry or immunohistochemistry) and genetics. 3 …”

Autoimmune Lymphoproliferative Syndrome in Children with Nonmalignant Organomegaly, Chronic Immune Cytopenia, and Newly Diagnosed Lymphoma

“…However, the median age of lymphoma patients was found as 12 years in our study. Most reported patients with ALPS have Hodgkin lymphoma, and EBV is positive in these cases [ 15 , 16 ]. Similarly, our patients were mostly diagnosed with Hodgkin lymphoma, but investigations of these patients revealed only two cases with EBV.…”

“…Autoimmune lymphoproliferative syndrome (ALPS) is characterized by nonmalignant organomegaly, immune cytopenia, and an increased risk for lymphoma, as well as mutation in the FAS -mediated apoptotic pathway [ 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 ]. Few studies have considered the identification of ALPS in certain populations, such as patients with Evans syndrome or lymphoma [ 11 , 12 , 13 , 14 , 15 , 16 ].…”