Document Detail


Prognostic significance of the BAALC isoform pattern and CEBPA mutations in pediatric acute myeloid leukemia with normal karyotype: a study by the Japanese Childhood AML Cooperative Study Group.
MedLine Citation:
PMID:  20495894     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
High BAALC (brain and acute leukemia, cytoplasmic) gene expression may indicate an adverse prognosis for adults who have acute myeloid leukemia (AML) and a normal karyotype, but its prognostic significance for pediatric AML cases is unclear. Whether different BAALC isoform patterns are of prognostic significance is also unclear. Newly diagnosed AML patients with normal karyotype who were treated by the Japanese Childhood AML Cooperative Treatment Protocol AML 99 were analyzed in terms of their BAALC expression levels (n = 29), BAALC isoforms (n = 29), and CEBPA mutations (n = 49). Eleven and 18 patients exhibited high and low BAALC expression, respectively, but these groups did not differ significantly in terms of overall survival (54.6 vs. 61.1%, P = 0.55) or event-free survival (61.4 vs. 50.0%, P = 0.82). Three of these 29 patients (10.3%) expressed the exon 1-5-6-8 BAALC isoform along with the expected 1-6-8 isoform and had adverse clinical outcomes. Novel CEBPA mutations were also identified in four of 49 patients (8.2%). All four patients have maintained complete remission for at least 5 years. Thus, 1-5-6-8 isoform expression may be associated with an adverse prognosis in pediatric AML with normal karyotype. CEBPA mutations may indicate a favorable prognosis.
Authors:
Yasuhiro Mizushima; Tomohiko Taki; Akira Shimada; Yoshihiro Yui; Yoshimi Hiraumi; Hiroshi Matsubara; Motonobu Watanabe; Ken-ichiro Watanabe; Yuri Kamitsuji; Yasuhide Hayashi; Ichiro Tsukimoto; Ryoji Kobayashi; Keizo Horibe; Akio Tawa; Tatsutoshi Nakahata; Souichi Adachi
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-05-22
Journal Detail:
Title:  International journal of hematology     Volume:  91     ISSN:  1865-3774     ISO Abbreviation:  Int. J. Hematol.     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-06-16     Completed Date:  2010-09-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9111627     Medline TA:  Int J Hematol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  831-7     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Graduate School of Medicine, Kyoto University, 54 Kawahara-cho, Syogoin, Sakyo-ku, Kyoto, 606-8507, Japan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Asian Continental Ancestry Group / genetics*
CCAAT-Enhancer-Binding Proteins / diagnostic use*,  genetics
Child
Child, Preschool
Female
Gene Expression Regulation, Leukemic*
Humans
Infant
Infant, Newborn
Karyotyping
Leukemia, Myeloid, Acute / diagnosis*,  genetics
Male
Mutation*
Neoplasm Proteins / diagnostic use*,  genetics
Prognosis
Protein Isoforms / diagnostic use,  genetics
Chemical
Reg. No./Substance:
0/BAALC protein, human; 0/CCAAT-Enhancer-Binding Proteins; 0/CEBPA protein, human; 0/Neoplasm Proteins; 0/Protein Isoforms

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