Résumé
Objective To analyze the clinical features and prognosis of pediatric acute myeloid leukemia (AML) with EVI1 gene positive.Methods The nested RT-PCR was performed to detect the EVI1 expression in pediatric AML patients from Jan.2009 to Dec.2011.The patients with EVI1 were investigated on clinical features,curative effects and prognosis.The differences between EVI1 (+) and EVI1 (-) patients were also analyzed.Results The frequency of EVI1 (+) expression was 15.65％ (13/83 cases)in pediatric AML,with the highest incidence in high-risk patients.EVI1 (+) was obviously associated with some unfavorable molecular genetic changes such as complex karyotype,MLL rearrangement,and monosomy 7.There were significant differences for EVI1 (+) group and EVI1 (-) group in the complete remission rate (45.5％ vs 79.3％,x2 =5.497,P ＜ 0.05) and complete remission(CR) rate after the first chemotherapy (18.2％ vs 63.8％,x2 =7.828,P ＜0.01).Although significant difference in death rate was not observed,EVI1(+) group had significantly higher early-death rate (45.5％ vs 8.6％,P ＜0.01).The EVI1(+) group also had lower 4 years event-free survival (EFS) [(21.2 ± 13.8) ％ vs (50.2 ± 9.1) ％,x2 =4.493,P ＜ 0.05] and lower 4 years overall survival(OS) [(32.4 ± 7.1) ％ vs (60.3 ± 10.9) ％,x2 =4.602,P ＜ 0.05] compared with EVI1 (-) group.But binary Logistic analysis did not identify EVI1 (+) as an independent unfavorable prognostic factor.Conclusions The pediatric AML with positive EVI1 expression had lower CR rate,higher early death rate and lower EFS.Positive EVI1 expression is related with an adverse outcome,but is not an independent poor prognostic factor.
Résumé
<p><b>OBJECTIVE</b>This study compared the differences in clinical features between chronic aplastic anemia (CAA) and myelodysplastic syndrome (MDS) in children in order to provide a basis for the differential diagnosis of the two diseases.</p><p><b>METHODS</b>A retrospective study of 23 cases of CAA and 9 cases of MDS from September 2007 to September 2010 was performed. The clinical data including routine blood test results, reticulocyte counts, serum lactate dehydrogenase level, serum ferritin level, cytological examination of bone marrow, bone marrow CD34+ cell counts, bone marrow chromosome and FISH test results were compared between the CAA and MDS groups.</p><p><b>RESULTS</b>Neutrophils, reticulocytes, and serum ferritin and lactate dehydrogenase levels increased in the MDS group compared with those in the CAA group. There were significant differences in bone marrow blast cell counts and dyshematopoiesis phenomena of three lines blood cells between the CAA and MDS groups. The bone marrow CD34+ cell counts and the rate of chromosomal abnormalities detected in bone marrow cytogenetic analysis in the MDS group were significantly higher than those in the CAA group.</p><p><b>CONCLUSIONS</b>There are differences in the results of laboratory examinations and morphological and cytogenetic examinations of bone marrow between the children with CAA and MDS. The differences are useful to the differential diagnosis of the two diseases.</p>