RESUMEN
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.
Asunto(s)
Linfoma no Hodgkin/mortalidad , Adolescente , Distribución por Edad , Brasil/epidemiología , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Estimación de Kaplan-Meier , Linfoma no Hodgkin/patología , Masculino , Estudios Retrospectivos , Distribución por SexoRESUMEN
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.
RESUMO OBJETIVO Descrever as características clínicas e demográficas de pacientes pediátricos com linfoma não Hodgkin (LNH) em uma unidade terciária de Hematologia Pediátrica entre 1982-2015. PACIENTES E MÉTODOS Estudo de coorte retrospectivo de dados de prontuários de 140 pacientes com idade até 16 anos com LNH. Características demográficas e dados relativos ao diagnóstico e evolução foram analisados. A sobrevida global (SG) e estratificada pelos subtipos histológicos mais frequentes foi analisada pelo método de Kaplan-Meier. RESULTADOS Dados de 136 pacientes com LNH de novo e quatro com LNH como segunda neoplasia foram analisados. A mediana de idade ao diagnóstico foi 6,4 anos (intervalo interquartil: 4,2 a 11,1 anos); 101 pacientes eram meninos. Onze pacientes apresentavam imunodeficiência (quatro primária, quatro secundária ao vírus da imunodeficiência humana adquirida, dois pós-transplante hepático e um com síndrome linfoproliferativa autoimune). Os tipos histológicos mais frequentes foram o LNH de células B madura (LNH-B, 67,1% dos pacientes), sendo o linfoma de Burkitt o subtipo mais frequente, e o linfoma linfoblástico (LL, 21,4%). A principal manifestação clínica ao diagnóstico foi massa abdominal (41,4%). A mediana de seguimento dos sobreviventes foi 7,7 anos (intervalo interquartil: 3,3 a 10,9 anos). Treze pacientes recidivaram (cinco alcançaram segunda remissão clínica), 35 faleceram, 103 permanecem vivos em remissão completa e dois perderam o seguimento. A probabilidade de SG em cinco anos foi 74,5%±3,8%. Para os pacientes com LL, LNH-B e os demais, a SG foi 80,4%±7,9%, 72,8%±4,7% e 74,5%±11%, respectivamente (P=0,58). CONCLUSÃO Nossos resultados são comparáveis aos de outros países de renda média.
Asunto(s)
Humanos , Masculino , Femenino , Preescolar , Niño , Adolescente , Linfoma no Hodgkin/mortalidad , Linfoma no Hodgkin/patología , Brasil/epidemiología , Estudios Retrospectivos , Estudios de Seguimiento , Distribución por Sexo , Distribución por Edad , Estimación de Kaplan-MeierRESUMEN
Neste estudo é relatado o quadro clínico de cinco crianças com linfoma não-Hodgkin secundário a imunodeficiência ou imunossupressão: três portadoras do vírus da imunodeficiência humana, uma com imunodeficiência primária e uma após transplante hepático. De acordo com a classificação atual, os tipos histológicos foram: linfoma linfoblástico de células B precursoras (2), linfoma cutâneo de grandes células anaplásico (1), linfoma de células B periféricas, sugestivo de Burkitt (1), e linfoma linfoblástico de células T precursoras (1). Todos os pacientes foram submetidos a quimioterapia, sendo que dois estão em remissão clínica, dois morreram e um continua em tratamento. Após a introdução da terapia anti-retroviral combinada e o aumento dos transplantes de órgãos sólidos ocorre maior risco de neoplasia nesses pacientes. Desse modo, é importante o seguimento desses pacientes para determinar os fatores de risco para o desenvolvimento de neoplasias e definir adequada estratégia de tratamento.
The outcomes of five children with non-Hodgkin's lymphomas associated with immunodeficiency or immunosuppression is reported: three children with HIV, one with primary immunodeficiency and one after liver transplantation. According to the REAL classification, two patients had precursor B-lymphoblastic lymphomas, one had an anaplastic large cell lymphoma, one had a peripheral B-cell neoplasm suggestive of Burkitt's lymphoma, and one had precursor T-lymphoblastic lymphoma. All patients received chemotherapy. Two are in complete remission, two died and one remains under treatment. There has been an increasing awareness of the risk of non-Hodgkin's lymphoma and lymphoproliferative disorders after active antiretroviral therapy and with the expansion of solid organ transplant programs in the pediatric setting. Thus, for these patients it is important to establish risk factors for hematological disorders and determine the optimal and safest treatment.