Cláudio Galvão de Castro Junior

Transplante de medula óssea e transplante de sangue de cordão umbilical em pediatria

Objective: to review the indications, main steps and complications of bone marrow transplantation in children. Sources: Medline-based literature review. Summary of the findings: we comment about the indications of autologous, allogeneic and syngeneic bone marrow transplantation, donor selections, harvest and infusion of the hematopoietic progenitor cells that will reconstitute the hematopoietic and immune systems. We describe the different conditioning regimens and the new sources of cells, such as cord blood. We also describe the most common events after the procedure, including infections, graft versus host disease, and cardiovascular, pulmonary, hepatic, genitourinary, and gastrointestinal complications. The late effects and their impact on quality of life are also discussed. Conclusions: bone marrow transplantation does not confer an absolutely normal life span to all the patients; however, it represents the only chance of cure for children with certain neoplastic or immunological diseases. By knowing the steps of the procedure, pediatricians can be a source of information on bone marrow transplantation to the patients and their families.

Oral vs. intravenous empirical antimicrobial therapy in febrile neutropenic patients receiving childhood cancer chemotherapy.

OBJECTIVE To compare the use of intravenous vs. oral antibiotic therapy. METHODS All febrile neutropenic patients younger than 18 years old with low risk of complications and receiving chemotherapy were selected. The study was conducted from 2002 to 2005 at the Pediatric Oncology Unit of Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. Patients were divided into group A and group B and were randomly assigned to receive oral or intravenous therapy. The empirical antimicrobial treatment used for group A consisted in oral ciprofloxacin plus amoxicillin-clavulanate and intravenous placebo, and group B received cefepime and oral placebo. RESULTS A total of 91 consecutive episodes of febrile neutropenia in 58 children were included in the study. For patients of group A, treatment failure rate was 51.2%; the mean length of hospital stay was 8 days (range 2-10 days). For patients treated with intravenous antibiotic therapy, treatment failure rate was 45.8%; the mean length of hospital stay was 7 days (range 3-10 days). CONCLUSION There was no difference in the outcome in oral vs. intravenous therapy. There is need of larger randomized trials before oral empirical therapy administered to this population should be considered the new standard of treatment.

Antibioticoterapia oral versus endovenosa em crianças neutropênicas febris recebendo quimioterapia

OBJECTIVE: To compare the use of intravenous vs. oral antibiotic therapy. METHODS: All febrile neutropenic patients younger than 18 years old with low risk of complications and receiving chemotherapy were selected. The study was conducted from 2002 to 2005 at the Pediatric Oncology Unit of Hospital de Clinicas de Porto Alegre, Porto Alegre, Brazil. Patients were divided into group A and group B and were randomly assigned to receive oral or intravenous therapy. The empirical antimicrobial treatment used for group A consisted in oral ciprofloxacin plus amoxicillin-clavulanate and intravenous placebo, and group B received cefepime and oral placebo. RESULTS: A total of 91 consecutive episodes of febrile neutropenia in 58 children were included in the study. For patients of group A, treatment failure rate was 51.2%; the mean length of hospital stay was 8 days (range 2-10 days). For patients treated with intravenous antibiotic therapy, treatment failure rate was 45.8%; the mean length of hospital stay was 7 days (range 3-10 days). CONCLUSION: There was no difference in the outcome in oral vs. intravenous therapy. There is need of larger randomized trials before oral empirical therapy administered to this population should be considered the new standard of treatment.

Autologous stem cell transplantation as first line treatment after incomplete excision of pancreatoblastoma.

Pancreatoblastoma is a rare tumor and surgery with complete resection is the main treatment approach. Prognosis for patients with residual disease after surgery is usually dismal. A 14-year-old girl with pancreatoblastoma in the pancreatic body and tail was submitted to preoperative chemotherapy. She underwent surgery and the tumor was resected with microscopic margins. Postoperative chemotherapy was followed by high dose chemotherapy and autologous hematopoietic stem cell transplantation. After four years she remains very well with no evidence of disease. This is the first case reported of pancreatoblastoma that was treated with autologous hematopoietic stem cell transplantation as first line treatment without radiotherapy at the site of the microscopic disease.

Leucemia mielomonocítica juvenil: relato de caso

A leucemia mielomonocitica juvenil (LMMJ) e uma doenca rara, que representa de 2%a 3% de todas as leucemias pediatricas. E uma doenca clonal de celulas da linhagem mieloide, que apresenta caracteristicas de mieloproliferacao e de displasia. Os sinais e os sintomas sao resultantes da infiltracao de celulas monociticas malignas em orgaos nao hematopoeticos. Os sintomas mais comuns sao febre, tosse, infeccao, fraqueza, palidez, linfadenopatia, hepatoesplenomegalia, lesoes cutâneas e manifestacoes hemorragicas. Como a LMMJ exibe um curso clinico muito agressivo e responde pobremente a quimioterapia, o transplante de celulas-tronco hematopoeticas e a unica modalidade terapeutica curativa. Neste estudo, relatamos o caso de um paciente do sexo masculino, com um ano e dez meses de idade, que compareceu na emergencia do Hospital de Clinicas de Porto Alegre por apresentar febre, com diagnostico previo de mononucleose feito em outra Instituicao. A apresentacao clinica, em conjunto com os achados laboratoriais, permitiu o diagnostico correto. O paciente foi tratado com quimioterapia e submetido a transplante de celulas-tronco hematopoeticas.

Analysis of predictive factors of complete blood count for a high-yield hematopoietic stem cell apheresis collection -

Introduction: In medical practice, quantification of peripheral blood CD34+ cells is a method of choice to calculate apheresis yield. Nevertheless, other predictive factors have been evaluated. The objective of this study was to investigate the association between CD34+ cells and number of apheresis collections from patients undergoing autologous bone marrow transplantation based on complete blood count parameters. Methods: Retrospective analysis of 113 patients of the Department of Pediatric Oncology of Hospital de Clinicas de Porto Alegre (HCPA) who had autologous bone marrow transplantation between 2004 and 2011 and underwent mobilization with granulocyte-colony stimulating factor in combination or not with chemotherapy. The following parameters were assessed: Total leukocyte count, platelets, hemoglobin, absolute neutrophil count, lymphocytes, monocytes, and immature granulocytes (IG). Statistical tests were used for asymmetric variables. Results: The correlation between CD34+ × 106/kg and leukocyte count (rs = 0.082; P = 0.394), platelets (rs = 0.078; P = 0.418), hemoglobin level (rs = −0.05; P = 0.564), neutrophils (rs = 0.042; P = 0.665), lymphocytes (rs = 0.048; P = 0.619), and IG (rs = 0.165; P = 0.083) revealed no significant result. In relation to monocytes, there was a weak but significant correlation (rs = 0.255; P = 0.007). In addition, patients with leukocyte count higher than 30 × 109/L and monocyte count higher than 1.8 × 109/L had good collection yield. Conclusion: Although there was no significant association between CD34 × 106/kg and blood parameters, we found that leukocyte count higher than 30 × 109/L and monocyte count higher than 1.8 × 109/L may be predictive factors of efficient collection. However, these values cannot be considered absolute factors because patients with lower counts also had satisfactory collections.

The rare association of leukoencephalopathy, cerebral calcifications, and cysts: case report

Although basal ganglia calcifications were described a long time ago,1,3,11 the association of leukoencephalopathy, cerebral calcifications, and cysts (LCC) is a very rare entity described in 1996.5 We present a new case of LCC and discuss clinical, neuroradiologic, and histopathologic findings regarding this association.