Predominance and characteristics of Burkitt lymphoma among children with non-Hodgkin lymphoma in northeastern Brazil

Abstract

The purpose of this paper was to define the histologic distrito the endemic (African) and sporadic forms of Burkitt lymbution, clinical features, and treatment response of childhood phoma, which differ in their clinical presentation, regional non-Hodgkin lymphoma (NHL) in northeastern Brazil. We incidence, association with Epstein-Barr virus, and biologic Patients and methodsThere was a striking predominance of the small noncleaved cell (Burkitt) subtype, which occurred in 92 of the 98 children and adolescents diagnosed with NHL. Subsequent analyses Between January 1980 and October 1987, a total of 98 chilfocused on these patients. The majority (n = 84) had advanced dren were diagnosed with non-Hodgkin lymphoma at the parental refusal/abandonment of therapy (10%). Epstein-BarrDiagnostic imaging studies were performed as indicated. virus (EBV) was detected in tumor cells from eight of the 11The pathologic diagnosis was established by histologic Of the 98 patients evaluated during the study period, 92 had small noncleaved cell histology. There were only five cases of lymphoblastic lymphoma and one of large cell histology. Having confirmed the predominance of Burkitt lymIntroduction phoma among the pediatric patients with NHL, we then focused on these 92 cases. Records were reviewed to evaluate The non-Hodgkin lymphomas of childhood are predominantly clinical and biological features, type of treatment, and outhigh-grade extranodal tumors that include the lymphoblastic, come. When sufficient paraffin-embedded tumor tissue was large cell, and small noncleaved cell subtypes. 1 The relative available, in situ RNA/RNA cytohybridization using plasmids frequency of these subtypes varies with geographic location.containing EBV-encoded small nuclear RNA was performed In the United States, the distribution is almost equal, 2 whereas at St Jude Children's Research Hospital to detect the presence small noncleaved cell tumors (Burkitt lymphoma) account for of Epstein-Barr virus DNA. 6 In these cases, the histologic diagthe majority of childhood NHLs in equatorial Africa. 3,4 In nosis was also reviewed and confirmed by a pediatric hematonortheastern Brazil -an area in which we have established a pathologist at St Jude (CWB). cooperative clinical program -anecdotal evidence suggests an increased overall incidence of lymphoma. However, to our knowledge, there have been no reports focusing on pediatric Hodgkin or non-Hodgkin lymphomas in this region. TreatmentWe noted an apparent predominance of the small noncleaved cell histology among children treated for NHL at a There were two distinct treatment periods. From 1980 to major pediatric cancer center in Recife, Brazil. In the present 1983, all consenting patients were treated with induction study, we evaluated the accuracy of this clinical impression chemotherapy similar to that used in the LSA 2 L 2 regimen and assessed long-term treatment outcome. We also sought to described by Wollner et al, 7 followed by maintenance therapy determine the relationship of the small noncleaved...

Disease patterns of pediatric non-Hodgkin lymphoma: A study from a developing area in Egypt

“…The present study describes the epidemiological and clinical characteristics of 142 Egyptian pediatric NHL patients admitted to two Pediatric Oncology Units. Patients aged <10 years consituted >88.5% of our study sample and there was a trend toward a decrease of the relative incidence with increasing age, with 88.5 and 11.5% for the age groups of 0-10 and >10 years, respectively, which was consistent with previously reported findings (16)(17)(18). Sandlund et al (1) and Manipadam et al (19) reported that the incidence of NHL increases uniformly with age and is rarely diagnosed before 2 years of age.…”

Linfoma não-Hodgkin na infância: características clínico-epidemiológicas e avaliação de sobrevida em um único centro no Nordeste do Brasil

“…We found evidence of a high prevalence of NHL of the Burkitt histological subtype (78.2%), when compared with American and European data. 3 Sandlund et al 6 have previously made the same observation with relation to the distribution of histological types, reporting a frequency of 94% of Burkitt's lymphoma in children with NHL in our region.…”

“…3 In our study we observed that NHL affects younger children than those described in foreign literature and this fact is compatible with other reports that have described other Brazilian experiences. 6,7,15,16 The possibility of infectious etiology, principally related to the Epstein-Barr virus, 4 being involved in the pathogenesis of Burkitt lymphomas may be a partial explanation for the emergence of this disease in younger patients in our region, since it is believed that populations in less privileged socioeconomic situations are exposed to infectious agents earlier.…”

“…3,5 In the northeast of Brazil, a high frequency of the Burkitt histological subtype has also been reported in the past. 6,7 Among the prognostic factors of childhood NHL that have been studied, the factor with greatest consensus in the literature is the tumor burden at the time of diagnosis, normally quantified by staging and serum lactate dehydrogenase (LDH) Serum LDH assays were performed within 24 hours of patient admission, being considered elevated when over 500 UI/dL (International Units per deciliter).…”

Prevalence of Epstein-Barr virus antibodies in healthy children and adolescents in Vitória, State of Espírito Santo, Brazil

“…Association of EBV infection with Burkitt's lymphoma and Hodgkin's disease in Brazil has been reported in Recife 22 , Salvador 2 3 and São Paulo 9 .…”