Shorter Maintenance Therapy in Childhood Acute Lymphoblastic Leukemia: The Experience of the Prospective, Randomized Brazilian GBTLI ALL-93 Protocol.

AIM: Maintenance therapy is an important phase of the childhood ALL treatment, requiring 2-year long therapy adherence of the patients and families. Weekly methotrexate with daily 6-mercaptopurine (6MP) constitutes the backbone of maintenance therapy. Reduction in the maintenance therapy could overweight problems related with poverty of children with ALL living in limited-income countries (LIC). OBJECTIVE: To compare, prospectively, the EFS rates of children with ALL treated according to two maintenance regimens: 18 vs. 24 months duration. MATERIALS AND METHODS: From October 1993 to September 1999, 867 consecutive untreated ALL patients <18 years of age were treated according to the Brazilian Cooperative Group for Childhood ALL Treatment (GBTLI) ALL-93 protocol. Risk classification was based exclusively on patient's age and leukocyte count (NCI risk group) and clinical extra medullary involvement of the disease. Data were analyzed by the intention-to-treat approach. RESULTS: Fourteen patients (1.6%) were excluded: wrong diagnosis (n = 7) and previous corticosteroid (n = 7). Of the 853 eligible patients, 421 were randomly allocated, at study enrollment, to receive 18-month (group 1) and 432 to receive 24-month (group 2) maintenance therapy. Complete remission rate was achieved in 96% of the patients (817/853). Twenty-eight patients (3.4%) died during the induction phase. Thirty-four patients (4.0%) were lost to follow-up. The overall EFS was 66.1 ± 1.7% at 15 years. No difference was seen according to maintenance: EFS15y was 65.8 ± 2.3% (group 1) and 66.3 ± 2.3% (group 2; p = 0.79). No difference between regimens was detected after stratifying the analyses according to factors associated with adverse prognosis in this study (age group <1 year or >10 years and high WBC at diagnosis). Overall death in remission rate was 6.85% (56 patients). Deaths during maintenance were 13 in group 1 and 12 in group 2, all due to infection. Over 15 years of follow-up, two patients both from group 2 presented a second malignancy (Hodgkin's disease and thyroid carcinoma) after 8.3 and 11 years off therapy, respectively. CONCLUSION: Six-month reduction of maintenance therapy in ALL children treated according to the GBTLI ALL-93 protocol provided the same overall outcome as 2-year duration regimen.

Linfomas não hodgkin em crianças e adolescentes um estudo Descritivo no estado da Bahia-Brasil / Non - Hodgkin lymphoma in children and adolescents: a descriptive Study in the state of Bahia - Brazil

O objetivo desta pesquisa foi investigar as características sócio-demográficas e clínico-patológicas dos linfomas não Hodgkin LNH em crianças e adolescentes no estado da Bahia. Do ponto de vista da metodologia, trata se de estudo descritivo com dados secundários de 111 pacientes com LNH, procedentes de dois centros de referência. Critérios de inclusão idade 20 anos, residência na Bahia e admissão no período de janeiro de 2000 a dezembro de 2005, com diagnóstico anátomo patológico de LNH. Obtiveram se os seguintes resultados: dos 111 pacientes, 76 68,5% presentaram idade entre cinco e 14 anos, com uma média de 8,6 4,3 anos. Linfomas de Burkitt LB predominaram 56,8%seguidos pelos linfomas linfo blásticos e linfomas de grandes células, cada um com 23 casos 20,7%. Sítio primário do tumor predominante foi abdominal 57,7 %, seguido por cabeça e pescoço 19,8%, mediastino 16,2% e outros 6,3%. Manifestações clínicas mais freqüentes nos pacientes com LB foram: dor abdominal 39,7%, aumento do volume abdominal 20,6%e presença de massa no abdome 12,7%. Quase 41% dos pacientes com LB apresentavam sintomas há mais de um mês. O valor médio da enzima lactato desidrogenase foi 1.896 4.277,6 U/L nos pacientes com LB. Os resultados apontam as características quepredominaram em pacientes com LNH pediátricos. Deve-se sempre cogitar sobre a presença dessa afecção como diagnóstico diferencial quando tais características estiverem presentes.

To investigate socio-demographic and clinical-pathological characteristics of the non-Hodgkin?s lymphoma NHL in children and adolescents in the State of Bahia. It is a descriptive study with secondary data from 111 patients with NHL, coming from two reference health centers. Criteria for inclusion: age 20 years, resident in Bahia, and admission from January 2000 to December 2005, with anatomic pathological diagnosis of NHL. Among the 111 patients, 76 68.5% were aged 5 14 years, with a mean of 8.6 4.3 years. The Burkitt lymphoma BL prevailed 56.8%, followed by lymphoblastic lymphoma LL and large cell lymphoma LCL, each with 23 cases 20.7%. Abdominal region was the dominant site 57.7%, followed by head and neck 19.8%, mediastinum 16.2% and other sites 6.3%. The most frequent clinical manifestations in patients with BL were: abdominal pain 39.7%, increase in the abdominal volume 20.6% and presence of mass in the abdomen 12.7%. Approximately 41% of patients with BL had symptoms one month before diagnosis. Average level of serum lactate dehydrogenase enzyme in patients with BL was 1,896 4,277.6 U L. These results show the characteristics that prevailed in pediatric patients with NHL. So, this disease should be thought of as differential diagnosis when these characteristics are present.

Significado prognóstico do tamanho tumoral no osteossarcoma infantil / Prognostic significance of tumor size in child osteogenic sarcoma

Entre 1995 e 1998, vinte e sete pacientes foram incluídos neste estudo clínico prospectivo. A idade variou entre 8,9 e 17,5 anos (média de 13,5), 20 (74 por cento) eram do sexo masculino e 7 (26 por cento) do sexo feminino com seguimento entre 3 e 39 meses (média de 17). O osteossarcoma incidiu no joelho (55,5 por cento), tornozelo (26 por cento), ombro (11,1 por cento), pelve (3,7 por cento) e quadril (3,7 por cento). Tumores menores que 12 cm eram considerados pequenos e maiores que 12 cm eram considerados grandes. Os pacientes foram submetidos a avaliação clínica, radiológica e histológica e classificados pelo sistema de Enneking (13 eram IIB e 8 eram IIIB); foram tratados com agentes quimioterápicos de acordo com o protocolo brasileiro 91 e pelo controle cirúrgico local. Foram realizadas 9 (42,9 por cento) cirurgias radicais e 12 (57,1 por cento) cirurgias de preservação do membro. De acordo com o tamanho tumoral, 8 (38,1 por cento) dos 14 (67,9 por cento) pacientes com tumores maiores que 12 cm faleceram; ao contrário, nenhum dos 7 pacientes com tumores menores que 12 cm apresentaram recidiva ou faleceram. Os autores concluem que a medida do tamanho tumoral pode vir a ser um bom fator prognóstico, sendo também de baixo custo, simples, de fácil reprodutibilidade e especialmente útil para tomada de decisões pré-tratamento.