RESUMO
Acquired hemophagocytic lymphohistiocitosis (HLH) syndrome can be a complication of visceral leishmaniasis (VL). A multicenter prospective study was conducted to determine the frequency of HLH syndrome in children with VL. Twenty-four children with VL were identified, and 10 (41%) developed HLH syndrome. VL should be ruled out in all children with HLH criteria living in or coming from endemic areas.
Assuntos
Leishmaniose Visceral/complicações , Linfo-Histiocitose Hemofagocítica/epidemiologia , Linfo-Histiocitose Hemofagocítica/patologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos ProspectivosAssuntos
Antineoplásicos/administração & dosagem , Benzamidas/administração & dosagem , Transplante de Células-Tronco Hematopoéticas , Piperazinas/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Pirimidinas/administração & dosagem , Adolescente , Criança , Esquema de Medicação , Seguimentos , Humanos , Mesilato de Imatinib , Análise de Sobrevida , Resultado do TratamentoAssuntos
Anemia Hipocrômica/genética , Proteínas de Transporte de Cátions/genética , Homozigoto , Sobrecarga de Ferro/genética , Mutação , Substituição de Aminoácidos , Anemia Hipocrômica/complicações , Criança , Éxons , Predisposição Genética para Doença , Humanos , Sobrecarga de Ferro/complicações , MasculinoRESUMO
Philadelphia-chromosome acute lymphoblastic leukaemia (Ph+ ALL) is a subgroup of ALL with very high risk of treatment failure. We report here the results of the Sociedad Española de Hematología y Oncología Pediátricas (SEHOP/SHOP) in paediatric Ph+ ALL treated with intermediate-dose imatinib concurrent with intensive chemotherapy. The toxicities and outcome of these patients were compared with historical controls not receiving imatinib. Patients with Ph+ ALL aged 1-18years were enrolled in three consecutive ALL/SHOP trials (SHOP-94/SHOP-99/SHOP-2005). In the SHOP-2005 trial, imatinib (260mg/m(2) per day) was given on day-15 of induction. Allogeneic haematopoietic stem-cell transplantation (HSCT) from a matched related or unrelated donor was scheduled in first complete remission (CR1). Forty-three patients were evaluable (22 boys, median age 6·8years, range, 1·2-15). Sixteen received imatinib whereas 27 received similar chemotherapy without imatinib. Seventeen of 27 and 15 of 16 patients in the non-imatinib and imatinib cohort, respectively, underwent HSCT in CR1. With a median follow-up of 109 and 39months for the non-imatinib and imatinib cohorts, the 3-year event-free survival (EFS) was 29·6% and 78·7%, respectively (P=0·01). These results show that, compared to historical controls, intermediate dose of imatinib given concomitantly with chemotherapy and followed by allogeneic HSCT markedly improved early EFS in paediatric Ph+ ALL.
Assuntos
Transplante de Células-Tronco Hematopoéticas/métodos , Piperazinas/administração & dosagem , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Pirimidinas/administração & dosagem , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Benzamidas , Criança , Pré-Escolar , Intervalo Livre de Doença , Feminino , Seguimentos , Humanos , Mesilato de Imatinib , Lactente , Masculino , Cromossomo Filadélfia , Piperazinas/toxicidade , Leucemia-Linfoma Linfoblástico de Células Precursoras/mortalidade , Pirimidinas/toxicidade , Espanha , Doadores de Tecidos , Transplante Homólogo , Resultado do TratamentoRESUMO
Juvenile granulosa cell tumor of the testis is an infrequent tumor of the gonadal stroma characteristic of the pediatric age. It usually appears as a scrotal mass and less frequently as an abdominal or inguinal mass. It may be associated with ambiguous genitalia and/or abnormal sex chromosomes. The recommended treatment is orchiectomy alone because local recurrence or metastasis have never been observed. We describe a patient with a juvenile granulosa cell tumor of the testis and review the literature.
