Original Article Keyla Christy Christine Mendes Sampaio Cunha Maria Christina Lopes Araujo Oliveira Ana Cecília Silva Gomes Lucia Porto Fonseca de Castro Marcos Borato Viana Universidade Federal de Minas Gerais – UFMG, Belo Horizonte, MG, Brazil Conflict-of-interest disclosure: The authors declare no competing financial interest Submitted: 4/17/2012 Accepted: 6/25/2012 Corresponding author: Maria Christina Lopes Araujo Oliveira Departamento de Pediatria - Unidade de Hematologia Pediátrica. Hospital das Clínicas, Universidade Federal de Minas Gerais – UFMG Av. Prof. Alfredo Balena, 190 30130-100 Belo Horizonte, MG, Brazil chrismariana@gmail.com www.rbhh.org or www.scielo.br/rbhh DOI: 10.5581/1516-8484.20120093 Rev Bras Hematol Hemoter. 2012;34(5):361-6 Clinical course and prognostic factors of children with Burkitt’s lymphoma in a developing country: the experience of a single centre in Brazil Objective: Burkitt’s lymphoma is the most common subtype of non-Hodgkin lymphoma in children. The aim of this study was to characterize the clinical course and prognostic factors of children and adolescents with Burkitt’s lymphoma treated in the Hematology Unit of Hospital das Clínicas, Universidade Federal de Minas Gerais (UFMG). Methods: A retrospective cohort study was made of 50 consecutive cases of children and adolescents aged 16 years or less with Burkitt’s lymphoma admitted between January 1981 and December 2007. Prognostic factors associated with death were evaluated using the Kaplan-Meier method and compared by the two-tailed log-rank test. Results: The median age at diagnosis was 4.7 years. Most patients had abdominal tumors (66.7%) and advanced disease (68.9%) at diagnosis. Thirty-eight patients (84.4%) achieved complete clinical remission and 33 (73.3%) were alive at the first remission. Twelve children (26.7%) died. The median follow-up was 35 months with the probability of overall survival being 73% (89.2% and 35.7% for patients with uric acid < 7 mg/dL and ≥ 7.0 mg/dL, respectively - p-value < 0.001). Uric acid was the only significant prognostic factor at diagnosis. Conclusion: Our findings confirm the favorable prognosis of children with Burkitt’s lymphoma even when treated with intermediate doses of methotrexate (500 mg/m2). Survival was significantly lower for individuals with concentrations of uric acid ≥ 7 mg/dL. Keywords: Burkitt’s lymphoma; Lymphoma, Non-Hodgkin; Child; Uric acid; Survival; Prognosis; Child Introduction The most frequent immunophenotype of non-Hodgkin lymphoma in children and adolescents is B-cell lymphomas (NHL-B). Burkitt’s lymphoma (BL) is the most common subtype, corresponding to approximately 40% of cases(1); it occurs worldwide and has endemic, sporadic and immunodeficiency-associated phenotypes(2). The endemic form classically presents as a jawbone tumor in children of African descendency. Its incidence is high at 50–100 cases per million during the first 15 years of life(3). Sporadic BL has a low incidence and occurs without specific geographic or climatic associations. In contrast to adults, lymph node involvement is less common among children with extranodal sites being the most frequent presentation(4). Immunodeficiency-associated BL mainly occurs in patients infected with human virus immunodeficiency (HIV) but also occurs in allograft recipients and in individuals with congenital immunodeficiencies(5). The pathogenesis of BL is universally associated with specific chromosomal translocations that lead to deregulation of the c-Myc oncogene. Other cellular genetic changes have been implicated in the pathogenesis including abnormalities of the p53-ARF pathways and environmental factors such as infection with Epstein-Barr virus (EBV) and malaria(6). The interactions between these cellular and environmental events continue to fascinate the scientific community(7). Among human neoplasms, BL has the shortest doubling time. Its unequalled proliferation rate creates special challenges for diagnosis and treatment(4). In addition, although BL is a highly aggressive lymphoma, 80% to 90% of children on risk-adapted chemotherapy are cured in the developed world(8,9). The aim of this study was to contribute to the knowledge about the clinical course and identification of prognostic factors related to death in 45 children and adolescents followed up at a single teaching hospital over a 26-year period. Methods In this retrospective cohort study, the medical charts of 50 consecutive cases of children and adolescents aged 16 years or less with de novo BL were reviewed. The patients were admitted to the Pediatric Hematology Unit, University Hospital, UFMG between January 1981 and December 2007. Five patients were excluded because of previous treatment at other institutions (n = 1), severe concomitant immunodeficiency (post-transplantation lymphoma; 361