RESUMEN
Variant Philadelphia (Ph) chromosome can be observed in 5-10 % of chronic myelogenous leukemia (CML) patients. However, there are only a few studies which have analyzed the prognostic implications of these complex translocations in CML patients after the advent of imatinib mesylate and the results found are conflicting. We investigated the clinical features and cytogenetic response of Brazilian chronic phase (CP) CML patients with variant Ph treated with imatinib mesylate. Among 93 CP CML patients, eight (8.6 %) exhibited complex translocations, involving one (n = 6), two (n = 1), or three (n = 1) additional chromosomes. At 6, 12, and 18 months, a complete cytogenetic response was observed in 100 % of variant Ph patients, respectively. No significant difference was found between variant Ph and standard translocation patients regarding the response to IM treatment at 6, 12, and 18 months. Likewise, there was no statistically significant difference between the two groups concerning the overall survival, failure-free survival, progression-free survival, and event-free survival. The results obtained in our study, despite our sample size, suggest, in agreement to other data found in the literature, that the presence of variant Philadelphia chromosome does not bestow a prognostic disadvantage when compared to the group with classic Ph. This observation does not suggest the need to adjust the treatment protocol due to the presence of variant Ph. However, further studies with larger sample sizes and evaluating both the cytogenetic and molecular response to IM treatment should be conducted to confirm our findings.
Asunto(s)
Antineoplásicos/uso terapéutico , Resistencia a Antineoplásicos/genética , Proteínas de Fusión bcr-abl/genética , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Cromosoma Filadelfia , Piperazinas/uso terapéutico , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirimidinas/uso terapéutico , Cariotipo Anormal , Adolescente , Adulto , Anciano , Benzamidas , Brasil/epidemiología , Cromosomas Humanos/ultraestructura , Supervivencia sin Enfermedad , Femenino , Humanos , Mesilato de Imatinib , Leucemia Mielógena Crónica BCR-ABL Positiva/sangre , Leucemia Mielógena Crónica BCR-ABL Positiva/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Translocación Genética , Resultado del Tratamiento , Adulto JovenRESUMEN
A Brazilian sample of 58 patients with acute lymphoblastic leukemia has been prospectively followed up with the objective of evaluating evolution of disease. Age ranged from 6 months to 16 years. Of the 58 patients, 11 were positive and 47 were negative for the ETV6/RUNX1 fusion (previously known as TEL/AML1). After a minimum follow-up period of 57 months, 2 of the ETV6/RUNX1(+) patients had died and 11 of the ETV6/RUNX1(-), for an overall survival of 77.6%. Among the 11 ETV6/RUNX1(+) patients (age range, 2 years to 13 years 7 months), all achieved a complete remission; the average overall survival was 64.2 months and the average event-free survival was 61.7 months. Among the 47 ETV6/RUNX1(-) patients, 4 did not have a complete remission; the average overall survival was of 60.8 months and the average event-free survival was 57.2 months. No significant difference was observed between overall survival and event-free survival, nor in any of the other data comparatively analyzed. The patients had a cure rate similar to that described in literature. In this small sample population, the presence of the ETV6/RUNX1 fusion did not identify statistical difference in prognosis.
Asunto(s)
Subunidad alfa 2 del Factor de Unión al Sitio Principal/genética , Proteínas de Fusión Oncogénica/genética , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/genética , Proteínas Proto-Oncogénicas c-ets/genética , Proteínas Represoras/genética , Brasil , Preescolar , Supervivencia sin Enfermedad , Femenino , Estudios de Seguimiento , Humanos , Inmunofenotipificación , Cariotipificación , Masculino , Pronóstico , Estudios Prospectivos , Inducción de Remisión , Análisis de Supervivencia , Factores de Tiempo , Proteína ETS de Variante de Translocación 6RESUMEN
Devido a importancia da Residencia Medica (RM) na formacao de profissionais especializados, este ttrabalho descreve o perfil do concurso para RM no HCPA em 1995 nas areas de Clinica Medica, Cirurgia Geral, Ginecologia Obstetricia e Pediatria. O estudo analisa as notas obtidas conforme os criterios de selecao utilizados neste hospital, que compreendem prova escrita (exame AMRIGS), curriculo e entrevista. A totalidade dos candidatos aprovados na Clinica Medica situou-se acima da mediana obtida entre todos os candidatos convocados por esta area, nos tres instrumentos de avaliacao. Na Cirurgia, o percentual de candidatos aprovados situados acima da mediana foi de 100 por cento para entrevista e analise de curriculo, enquanto que na Ginecologia Obstetricia observou-se o mesmo somente no criterio entrevista e, na Pediatria, no curriculo. Esses dados sugerem que os intrumentos de selecao, particularmente a realizacao de entrevista e analise de curriculo, estao sendo utilizados com intensidades diferentes nadeterminacao da aprovacao dos candidatos