RESUMO
A patient with Turner's syndrome was found to have generalized infantile myofibromatosis with visceral involvement at birth. The infant was treated with interferon-alpha because of the size of the lesions. Two months after treatment, the lesions appeared to have decreased in size and showed evidence of maturation with decreased apoptosis on histologic examination. Interferon-alpha treatment might induce regression of myofibromatosis.
Assuntos
Interferon-alfa/administração & dosagem , Miofibromatose/terapia , Síndrome de Turner/terapia , Diagnóstico por Imagem , Feminino , Humanos , Lactente , Recém-Nascido , Injeções Subcutâneas , Interferon alfa-2 , Miofibromatose/diagnóstico , Miofibromatose/genética , Proteínas Recombinantes , Síndrome de Turner/diagnóstico , Síndrome de Turner/genéticaRESUMO
Familial erythrophagocytic lymphocytosis (FEL) is a rare, nonmalignant class II histiocytosis characterized by fever, irritability, hepatosplenomegaly, pancytopenia, and hemophagocytosis. Various chemotherapeutic regimens have had mixed success, with the only curative therapy being bone marrow transplantation. We report our experience with two children whose therapy with etoposide and steroids failed. They were successfully treated and had durable remissions with cyclosporine A (CSA). We propose that in FEL there may exist abnormal interactions between antigen-presenting cells and T-lymphocyte subsets, and that CSA may down-modulate this aberrant response. The use of a low-dose CSA regimen may represent a treatment option that should be further explored.
Assuntos
Ciclosporina/uso terapêutico , Histiocitose de Células não Langerhans/tratamento farmacológico , Histiocitose de Células não Langerhans/genética , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Transplante de Medula Óssea , Feminino , Histiocitose de Células não Langerhans/terapia , Humanos , Lactente , MasculinoAssuntos
Varicela/complicações , Esplenopatias/etiologia , Infarto do Baço/etiologia , Abdome , Dor nas Costas/etiologia , Varicela/sangue , Criança , Inclusões Eritrocíticas/patologia , Feminino , Humanos , Tolerância Imunológica , Leucemia Linfoide/complicações , Dor/etiologia , Cintilografia , Baço/diagnóstico por imagemRESUMO
A 28-month prospective study of 54 leukemic children was carried out to determine the incidence and type of infection associated with febrile episodes. Fever was caused by infections in 84 of 199 episodes (71%). Two-thirds of the febrile episodes and 57% of the documented infections occurred when leukemic activity was demonstrable. However, only nine of 29 febrile episodes which occurred at the time of initial diagnosis of acute leukemia were due to infection. All serious bacterial infections occurred in children with absolute granulocyte counts less than 500/mm3. Septicemia was responsible for seven of the 17 deaths which occurred during the period of observation. The five children with Pseudomonas infections were colonized 10 to 30 days before they developed their infection. The majority of viral infections occurred in patients in remission, and were principally caused by cytomegalovirus, varicella-zoster virus, or Epstein-Barr virus. With the exception of one patient who died with a complex infection (CMV and Pneumocystis carinii), the children in this study responded well to viral infections.