RESUMO
An association of deletions in the IKZF1 gene (IKZF1del) with poor prognosis in acute lymphoblastic leukemia (ALL) has been demonstrated. Additional deletions in other genes (IKZF1plus) define different IKZF1del subsets. We analyzed the influence of IKZF1del and/or IKZF1plus in the survival of children with ALL. From October 2009 to July 2021, 1055 bone marrow samples from patients with ALL were processed by Multiplex ligation-dependent probe amplification (MLPA). Of them, 28 patients died during induction and 4 were lost-in-follow-up, resulting in an eligible 1023 cases. All patients were treated according to ALLIC-BFM-2009-protocol. Patients were classified into three subsets: IKZF1not-deleted (IKZFF1not-del), IKZF1deleted (IKZF1del) and IKZF1del plus deletion of PAX5, CDKN2A, CDKN2B and/or alterations in CRLF2 with ERG-not-deleted (IKZF1plus). The LFSp and SE were calculated with the Kaplan−Meier calculation and compared with a log-rank test. From the 1023 eligible patients, 835 (81.6%) were defined as IKZF1not-del, 94 (9.2%) as IKZF1del and 94 (9.2%) as IKZF1plus. Of them, 100 (9.8%) corresponded to Standard-Risk (SRG), 629 (61.5%) to Intermediate-Risk (IRG) and 294 (28.7%) to High-Risk (HRG) groups. LFSp(SE) was 7 5(2)% for IKZF1not-del, 51 (6)% for IKZF1del and 48 (6)% for IKZF1plus (p-value < 0.00001). LFSp(SE) according to the risk groups was: in SRG, 91 (4)% for IKZF1not-del, 50 (35)% IKZF1del and 100% IKZF1plus (p-value = ns); in IRG, 77 (2)% IKZF1not-del, 61 (10)% IKZF1del and 54 (7)% IKZF1plus (p-value = 0.0005) and in HRG, 61 (4)% IKZF1not-del, 38 (8)% IKZF1del and 35 (9)% IKZF1plus (p-value = 0.0102). The IKZF1 status defines a population of patients with a poor outcome, mainly in IRG. No differences were observed between IKZF1del versus IKZF1plus. MLPA studies should be incorporated into the risk-group stratification of pediatric ALL.
RESUMO
INTRODUCCIÓN: En Argentina, la mortalidad por enfermedades malignas en edad pediátrica ocupa un lugar relevante y sus causas todavía no han sido estudiadas en el país. OBJETIVO: Analizar las tasas, causas y etapas de los fallecimientos relacionados con neoplasias en centros públicos seleccionados, desde enero de 2000 a diciembre de 2010. MÉTODOS: Se analizaron las historias clínicas de los pacientes fallecidos por cáncer en centros registrados en el Registro Oncopediátrico Hospitalario Argentino (ROHA) y en los registros individuales de los servicios de Hemato-Oncología. Se clasificaron las causas de mortalidad, la etapa en la cual se produjo el óbito y su relación con el tratamiento o con la patología de base. Se pesquisaron las causas de comorbilidad y las demoras en el diagnóstico y tratamiento. RESULTADOS: En 13 centros se analizó exitosamente un promedio >70...
INTRODUCTION: In Argentina, the mortality of pediatric malignant diseases occupies an important place causes have not yet been studied in the country. OBJECTIVE:To analyze mortality rates, causes and moment of death related to neoplasias in selected public centers from January 2000 until December 2010. METHODS: The analysis was conducted in clinical records of patients who died due to cancer. The cases were registered in the Argentine Hospital Oncopediatric Registry (ROHA)and by different registries belonging to hemato-oncological departments. Mortality causes were classified according to the phase of therapy when the event occurred and the relation shipof death with the treatment or underlying disease. Causes of comorbility and delays in diagnosis/treatment were also analyzed. RESULTS: In 13 centers, more than 70...