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.

[Gastrinoma has an infrequent prevalence in pediatric age: a case report]. / Gastrinoma tem ocorrência incomum na faixa etária pediátrica. Relato de um caso.

The authors present the clinical, laboratory and radiological findings suggestive ofgastrinoma in a patient 1 year and 9 months old. Laboratory tests obtained after fasting revealed elevated serum gastrin, supporting the suspected diagnosis of gastrinoma. In the endoscopy an elevated lesion with central depression was observed. The immunohistochemical examination revealed the benign nature of the tumor and the hyperplasia of argentaffin cells. Gastrinoma is a rare disease that predominantly affects young adults, but it must be considered in the pediatric group when clinical and laboratorial features of this disease are observed.

SIL-TAL1 fusion gene negative impact in T-cell acute lymphoblastic leukemia outcome.

SIL-TAL1 fusion gene and the ectopic expression of HOX11L2 are common molecular abnormalities in T-cell acute lymphoblastic leukemia (T-ALL). To verify their influence on outcome, we analyzed a Brazilian pediatric T-ALL series of cases. One hundred and ninety two children, age ranged 0-21 years old, were consecutively diagnosed and treated. Reverse transcriptase-polymerase chain reaction (RT-PCR) technique was used to identify the molecular alterations. Kaplan-Meyer method was applied to estimate overall survival. The most frequent maturation stage was T-IV (40.1%), and 30.7% of cases were CD10(+). SIL-TAL1(+) and HOX11L2(+) accounted for 26.7% and 10.3% of the cases, respectively. The overall survival (OS) was 74% in 80-month follow-up. HOX11L2(+) was not predictive factor for outcome. Considering patients younger than nine years-old, those with SIL-TAL1(+) presented a poorer outcome (p = 0.02). The results of this study suggest that in the Brazilian population only the presence of SIL-TAL1 can predict outcome in a restricted group of patients.

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.

Detection of mutations in GATA1 gene using automated denaturing high-performance liquid chromatography and direct sequencing in children with Down syndrome.

Denaturing high-performance liquid chromatography (dHPLC) was developed to screen DNA variations by separating heteroduplex and homoduplex DNA fragments by ion-pair reverse-phase liquid chromatography. In this study, we have evaluated the dHPLC screening method and direct sequencing for the detection of GATA1 mutations in peripheral blood and bone marrow aspirates samples from children with Down syndrome (DS). Cases were ascertained consecutively as part of an epidemiological study of DS and hematological disorders in Brazil. A total of 130 samples corresponding to 115 children with DS were analysed using dHPLC and direct sequencing methods to detect mutations in GATA1 exons 2, 3 and 4 gene sequences. The overall detection rate of sequencing and dHPLC screening methods was similar. Twenty mutations were detected in exon 2 and one mutation in exon 3 (c.231_232 dupGT) sequences of acute megakaryoblastic leukemia and transient leukemia samples. Four GATA1 mutations were newly described [c.155C > G; c.156_178 del23 bp; c.29_30 del GG; c.182C > A and c.151A > T,c.153_162 del 10 bp). Out of four, three had single nucleotide change. In conclusion, our results indicate that dHPLC is an efficient and valuable tool for GATA1 mutational analysis.

Cisplatin and etoposide in childhood germ cell tumor: brazilian pediatric oncology society protocol GCT-91.

PURPOSE: In 1988, we formed a consortium of Brazilian institutions to develop uniform standards for the diagnostic assessment and multidisciplinary treatment of children and adolescents with germ cell tumors. We also implemented the first childhood Brazilian germ cell tumor protocol, GCT-91, evaluating two-agent chemotherapy with cisplatin and etoposide (PE). We now report on the clinical characteristics and survival of children and adolescents with germ cell tumors treated on this protocol. PATIENTS AND METHODS: From May 1991 to April 2000, 115 patients (106 assessable patients) were enrolled onto the Brazilian protocol with a diagnosis of germ cell tumor. RESULTS: Patients were treated with surgery only (n = 35) and chemotherapy (n = 71). Important prognostic factors included stage (P = .025), surgical procedure at diagnosis according to resectability (P < .032), and abnormal lactate dehydrogenase value at diagnosis (P < .001). CONCLUSION: The improvement in survival by the introduction of a standard protocol is an important achievement. This is of particular importance for smaller institutions with previous limited experience in the treatment of childhood germ cell tumors. In addition, the results of a two-agent regimen with PE were favorable (5-year overall survival rate is 83.3% for patients in the high-risk group [n = 36] who received PE v 58.8% for patients in the high-risk patients group who received PE plus ifosfamide, vinblastine, and bleomycin [n = 17; P = .017]). Thus for selected patients, complex three-agent regimens may not be necessary to achieve long-term survival, even for some patients with advanced disease.

Gastrointestinal manifestations as initial presentation of acute leukemias in children and adolescents.

OBJECTIVE: this study aimed to determine the prevalence and characteristics of gastrointestinal manifestations on initial clinical presentation of acute leukemias (AL) in childhood. MATERIAL AND METHODS: this is a retrospective and descriptive study that assessed medical records of 354 patients with AL from January 1995 to December 2004. RESULTS: acute lymphoid leukemia (ALL) was diagnosed in 273 (77.1%) patients and acute non-lymphocytic leukemia (AML) in 81 (22.9%). There were 210 males (59.4%) and 144 females (40.6%). The most common presenting features were: abdominal pain (19.5% in ALL and 11.8% in AML), nausea and vomiting (14.9 in ALL and 14% in AML), abdominal distention (18.5 in ALL and 8.6% in AML; p 0.024), constipation (5% in ALL and 6.5% in AML), diarrhea (3.6% in ALL and 11.8% in AML; p 0.03%), and gastrointestinal bleeding (7.9% in ALL and 9.7% in AML). Ultrasound scanning was made in 61.1% and hepatomegaly was found on 33.6% and esplenomegaly on 28.5% of the patients with AL. Seventy-seven (21.7%) and 15 (4.2%) patients received nonsteroidal anti-inflammatory drugs and glucocorticoids before the diagnostic of AL. An association is well-defined between abdominal symptoms like nausea, vomiting and pain and use of this therapy but this association did not occurred clearly in this study. CONCLUSIONS: gastrointestinal symptoms are not very well-documented as initial manifestation of leukemia in children and should be considered on the differential diagnosis of gastrointestinal symptoms of unknown etiology in children.

Gastrointestinal manifestations as initial presentation of acute leukemias in children and adolescents / Manifestaciones gastrointestinales como presentación de las leucemias agudas en niños y adolescentes

OBJECTIVE: this study aimed to determine the prevalence and characteristics of gastrointestinal manifestations on initial clinical presentation of acute leukemias (AL) in childhood. MATERIAL AND METHODS: this is a retrospective and descriptive study that assessed medical records of 354 patients with AL from January 1995 to December 2004. RESULTS: acute lymphoid leukemia (ALL) was diagnosed in 273 (77.1%) patients and acute non-lymphocytic leukemia (AML) in 81 (22.9%). There were 210 males (59.4%) and 144 females (40.6%). The most common presenting features were: abdominal pain (19.5% in ALL and 11.8% in AML), nausea and vomiting (14.9 in ALL and 14% in AML), abdominal distention (18.5 in ALL and 8.6% in AML; p 0.024), constipation (5% in ALL and 6.5% in AML), diarrhea (3.6% in ALL and 11.8% in AML; p 0.03%), and gastrointestinal bleeding (7.9% in ALL and 9.7% in AML). Ultrasound scanning was made in 61.1% and hepatomegaly was found on 33.6% and esplenomegaly on 28.5% of the patients with AL. Seventy-seven (21.7%) and 15 (4.2%) patients received nonsteroidal anti-inflammatory drugs and glucocorticoids before the diagnostic of AL. An association is well-defined between abdominal symptoms like nausea, vomiting and pain and use of this therapy but this association did not occurred clearly in this study. CONCLUSIONS: gastrointestinal symptoms are not very well-documented as initial manifestation of leukemia in children and should be considered on the differential diagnosis of gastrointestinal symptoms of unknown etiology in children.

Objetivo: el objetivo del estudio fue determinar la prevalencia y las características de las manifestaciones gastrointestinales en la presentación clínica inicial de las leucemias linfoides agudas (LLA) en la infancia. Materialy métodos: se trata de un estudio descriptivo y retrospectivo que evaluó los registros médicos de 354 pacientescon LLA de enero de 1995 a diciembre de 2004. Resultados: la (LLA) ha sido diagnosticada en 273 (77,1%) pacientes y leucemia mieloide aguda (LMA) en 81 (22,9%). Hubo 210 niños (59,4%) y 144 niñas (40,6%). Los síntomas más comunes de presentaciónhan sido los siguientes: dolor abdominal(19,5% en LLA y 11,8% en el LMA), náuseas y vómitos (14,9 en LLA y 14% en LMA, P 0.024), distensión abdominal (18,5 en LLA y 8,6% en LMA, p 0,024), estreñimiento (5% en LLA y 6,5% en LMA), diarrea (3,6% en LLA y 11,8% en LMA, p 0,03%) y hemorragia gastrointestinal (7,9% en LLA y 9,7% enLMA). La ecografía fue realizada en 61,1% de los pacientes encontrándose hepatomegalia en 33,6% y esplenomegalia en 28,5% con LLA. Setenta y siete (21,7%) y 15 (4,2%) pacientes recibieron los fármacos antiinflamatorios no esteroides y glucocorticoides antes del diagnóstico de LLA. Hay una asociación bien definidaentre síntomas abdominales como náuseas, vómitos y dolor y el uso de esta terapia pero esta asociación no seprodujo claramente en este estudio. Conclusiones: las manifestaciones gastrointestinales no están bien documentadas como manifestaciones iniciales de la leucemia en los niños y debe considerarse en el diagnóstico diferencial de los síntomas gastrointestinales de etiología desconocida en estas edades.

Osteoarticular manifestations as initial presentation of acute leukemias in children and adolescents in Bahia, Brazil.

OBJECTIVE: This study was to determine the prevalence and characteristics of the osteoarticular manifestations on initial clinical presentation of acute leukemias (ALs) on childhood in the state of Bahia, Brazil. MATERIALS AND METHODS: This retrospective study assessed the medical records of 406 patients with AL from January 1995 to December 2004. RESULTS: Acute lymphocytic leukemia (ALL) was diagnosed in 313 (77.1%) patients and acute myeloid leukemia (AML), in 93 (22.9%) patients, including 241 males (59.4%) and 165 females (40.6%). Age ranged from 9 months to 15 years (average: 6.18 y). The most common presenting features were fever (18.5%), musculoskeletal diffuse tenderness (15.0%), pallor (11.4%), and leg tenderness (5.7%). Prior referral to our center, the most frequent initial diagnosis was anemia (15.8%), leukemia (15.0%), amygdalitis (3.7%), and rheumatic fever (2.7%). Osteoarticular manifestations were found on 54.7% of the patients with AL, with a higher frequency among patients between 1 and 9 years of age (58.7%, P=0.0007). The presence of joint tenderness (16.2% in ALLx5.4% in AML), arthritis (26.6% in ALLx9.7 in AML), bone tenderness (26.1% in ALLx16.1% in AML), limb tenderness (49.5% in ALLx25.8% in AML), and antalgic gait (32.8% in ALLx9.7% in AML) had higher prevalence on ALL. The large joints, chiefly the knees (10.6%), ankles (9.4%), elbows (4.4%), and shoulders (3.6%) were more often affected. CONCLUSIONS: AL should be considered on the differential diagnosis of osteoarticular symptoms of unknown etiology in children.

New guidelines for the clinical management of febrile neutropenia and sepsis in pediatric oncology patients.

OBJECTIVES: To provide a foundation for the diagnostic, prophylactic and therapeutic management of febrile neutropenia and sepsis in children with oncological diseases, with special attention to new protocols and guidelines. SOURCES: A review of the scientific literature utilizing an electronic bibliographic search on MEDLINE, Medscape, SciELO, Google, Cochrane and PubMED using the keywords febrile, neutropenic, cancer, children, sepsis, intensive, care. Articles published between 1987 and 2007 were selected, with preference given to review articles, protocols, systematic reviews, epidemiological studies, task force recommendations and phase III clinical trials. Consensus documents published by the Infectious Diseases Society of America, the Center for Diseases Control and the Infectious Diseases Working Party of the German Society of Hematology and Oncology, in addition to the recommendations of the World Federation of Pediatric Intensive and Critical Care Societies and Society of Critical Care Medicine, were also reviewed. SUMMARY OF THE FINDINGS: The use of aggressive chemotherapy regimens, bone marrow transplantation and intensive care resources have increased the survival rates of children with cancer and also their infectious morbidity, with septic complications as the principal cause of mortality. Several risk factors have been identified, such as neutropenia, oncology type, clinical signs and inflammatory response markers (polymerase chain reaction, procalcitonin) and also increased resistance to antimicrobials and antifungal agents. Protocols for risk classification, diagnosis and treatment should be established at each service, taking into account the microbiological flora of each population. Pediatric intensive care has increased the short and long-term survival of these patients. CONCLUSIONS: Oncology patients are particularly vulnerable to infectious complications. Early identification and treatment are fundamental to improving survival rates.

Novas diretrizes na abordagem clínica da neutropenia febril e da sepse em oncologia pediátrica / New guidelines for the clinical management of febrile neutropenia and sepsis in pediatric oncology patients

OBJETIVOS: Fornecer subsídios à abordagem diagnóstica, profilática e terapêutica da neutropenia febril e da sepse em criança com doença oncológica, dando especial atenção aos novos protocolos e diretrizes. FONTES DE DADOS: Revisão de literatura científica utilizando uma busca bibliográfica eletrônica nas páginas do MEDLINE, Medscape, SciELO, Google, Cochrane e PubMED com as palavras-chave febrile, neutropenic, cancer, children, sepse, intensive, care. Foram selecionados artigos publicados entre 1987 e 2007, preferencialmente artigos de revisão, protocolos, revisões sistemáticas, estudos epidemiológicos, recomendações de força-tarefa e ensaios clínicos fase III. Foram revistos os consensos publicados pela Infectious Diseases Society of America, Center for Diseases Control e Infectious Diseases Working Party da German Society of Hematology and Oncology, além de recomendações da World Federation of Pediatric Intensive and Critical Care Societies e da Society of Critical Care Medicine. SíNTESE DOS DADOS: A utilização de esquemas quimioterápicos agressivos, transplante de medula óssea e recursos de terapia intensiva aumentaram a sobrevida nas crianças com câncer e também a morbidade infecciosa, sendo as complicações sépticas a principal causa de mortalidade. Diversos fatores de risco têm sido identificados, como neutropenia, tipo oncológico, sinais clínicos e marcadores de resposta inflamatória (reação em cadeia da polimerase, procalcitonina), assim como a maior resistência aos antimicrobianos e antifúngicos. Protocolos de classificação de risco, de diagnóstico e tratamento devem ser estabelecidos em cada serviço, respeitando a flora microbiológica da população estudada. A terapia intensiva pediátrica tem aumentado a sobrevida a curto e longo prazo nestes pacientes. CONCLUSÕES: Pacientes oncológicos são particularmente vulneráveis a complicações infecciosas. A identificação e o tratamento precoce são fundamentais para a melhora da sobrevida.

OBJECTIVES: To provide a foundation for the diagnostic, prophylactic and therapeutic management of febrile neutropenia and sepsis in children with oncological diseases, with special attention to new protocols and guidelines. SOURCES: A review of the scientific literature utilizing an electronic bibliographic search on MEDLINE, Medscape, SciELO, Google, Cochrane and PubMED using the keywords febrile, neutropenic, cancer, children, sepsis, intensive, care. Articles published between 1987 and 2007 were selected, with preference given to review articles, protocols, systematic reviews, epidemiological studies, task force recommendations and phase III clinical trials. Consensus documents published by the Infectious Diseases Society of America, the Center for Diseases Control and the Infectious Diseases Working Party of the German Society of Hematology and Oncology, in addition to the recommendations of the World Federation of Pediatric Intensive and Critical Care Societies and Society of Critical Care Medicine, were also reviewed. SUMMARY OF THE FINDINGS: The use of aggressive chemotherapy regimens, bone marrow transplantation and intensive care resources have increased the survival rates of children with cancer and also their infectious morbidity, with septic complications as the principal cause of mortality. Several risk factors have been identified, such as neutropenia, oncology type, clinical signs and inflammatory response markers (polymerase chain reaction, procalcitonin) and also increased resistance to antimicrobials and antifungal agents. Protocols for risk classification, diagnosis and treatment should be established at each service, taking into account the microbiological flora of each population. Pediatric intensive care has increased the short and long-term survival of these patients. CONCLUSIONS: Oncology patients are particularly vulnerable to infectious complications. Early identification and treatment are fundamental to improving...

The high frequency of EBV infection in pediatric Hodgkin lymphoma is related to the classical type in Bahia, Brazil.

Pediatric Hodgkin lymphoma (HL) occurring in developing regions is different from HL in industrialized countries due to the higher frequency of association with Epstein-Barr virus (EBV) infection. This infection is related to classical HL (cHL) but is virtually absent in nodular lymphocyte predominant HL (nLPHL). We studied the phenotype and the expression of EBV gene products in 90 pediatric cases by immunohistochemistry and in situ hybridization. EBV-positive tumor cells were found exclusively in cHL. The infection occurred with high frequency in all cHL subtypes, but it predominated in the mixed cellularity and lymphocyte depletion subtypes. These results reinforce the hypothesis that EBV plays a major role in the etiology of pediatric cHL in developing areas. Curiously, the frequency of EBV infection in HL was identical to the previously described for Burkitt's lymphoma in the same pediatric population. As both lymphomas have a postulated precursor cell in the germinal center (GC), the pattern of latently EBV-infected GC cells previously described in Bahia may be related to the development of these lymphomas.

Biological diversity variations of pediatric acute leukemia in Brazil: contribution of immunophenotypic profiles to epidemiological studies

Os autores descrevem as características biológicas de 1.459 crianças com leucemias agudas no Brasil, para comparar os efeitos de diferentes perfis imunofenotípicos com fatores ambientais que podem estar associados à etiologia das leucemias linfoblásticas agudas (LLA). As classificações morfológicas e imunofenotípicas combinadas foram aplicadas em 96% dos casos. Nestes, 55% foram classificados como LLA de células B precursoras (LLA-Bp) que compreendem LLA-pro-B e LLA-comum, 15% LLA-T, e 1,6% LLA-B. A proporção de LLA-Bp e LLA-T difere entre si quanto à raça, com 59% das LLA-Bp em crianças brancas, enquanto 60,7% LLA-T em crianças não-brancas. No entanto, as análises proporcionais de brancos versus não brancos para cada subtipo, quando ajustadas por idade, são semelhantes em crianças maiores de 6 anos (60,3% LLA-Bp e 59,3% LLA-T), mas diferem substancialmente em crianças menores, com 63,6% de LLA-Bp e 37,3% de LLA-T em brancos (0,0001). Estes resultados são consistentes com excesso de LLA-Bp em crianças brancas mais jovens, embora a distribuição entre LLA-Bp e LLA-T em cada região seja semelhante sem significado estatístico. As taxas de incidências de LLA calculadas para cada região variaram de 2,2, 2,6 e 3,3/105 casos por ano para Bahia, Rio de Janeiro e Brasília, respectivamente. Para avaliar se o pico de incidência observado de LLA-Bp estaria relacionado com incidência de infeção viral, nós observamos que LLA-Bp apresentou uma curva ascedente de casos no verão e inverno, enquanto LLA-T apresentou pico de incidência no outono. Este estudo adiciona informações sobre epidemiologia de leucemias agudas no Brasil, no qual sugere que o subtipo LLA-comum poderia estar associado com tempo de exposição a infecção viral requerendo futuras análises específicas.

Acute leukemia with natural killer cells antigens in Brazilian children.

Malignancies arising from Natural Killer cells (NK) were described mainly comprising disorders related to large granular lymphocyte expansion. Clinical, morphological and immunophenotypic characteristics of myeloid/NK cell precursor acute leukemia (M/NK-AL) have been mainly described in adult patients. We identified nine children with M/NK-AL precursor cells in a series of 264 childhood acute leukemia diagnosed in our laboratory from consecutive referrals before treatment. CD34 +/-, CD13/33 +, CD11b +, a-MPO + and CD56 +, characterized these cases with TcR alphabeta and gammadelta negative cells. The differential diagnosis between subtypes of NK leukemia with those of lymphoid or myeloid origin in childhood raises the question regarding the pathogenesis and therapeutic implications of this rare entity in children.

Rhabdomyosarcoma mimicking Wilm's tumor

É apresentado um caso de rabdomiossarcoma pleomórfico em criança de seis anos envolvendo simultaneamente a musculatura da perna direita e o rim homolateral. O tumor renal distorcia o sistema pielocalicial e teve diagnóstico clínico de tumor de Wilms. Os autores discutem acêrca do possível sítio primário do tumor

A criança com câncer: o seu direito à vida e ao viver / The child with cancer: its right of life and to be alive

A autora mostra a situaçäo da criança com câncer no Brasil de hoje, as dificuldades para o seu diagnóstico, o seu encaminhamento adequado e o seu tratamento especializado. Mostra as medidas tomadas para sobrepujar estas dificuldades e as perspectivas futuras