Source:http://linkedlifedata.com/resource/pubmed/id/16614245
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| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
4
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| pubmed:dateCreated |
2006-8-7
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| pubmed:abstractText |
Activating mutations of the transmembrane receptor NOTCH1 are common in precursor T-cell lymphoblastic leukemia (T-ALL). We systematically analyzed the impact of activating NOTCH1 mutations on early treatment response and long-term outcome in 157 patients with T-ALL of the pediatric ALL-Berlin-Frankfurt-Munster (BFM) 2000 study. We confirm previous results that NOTCH1 mutations occur in more than 50% of T-ALL in children. In 82 patients (82/157; 52.2%), activating NOTCH1 mutations were identified either in the heterodimerization (55/82; 67.1%), in the PEST (13/82; 15.9%), or in both domains (14/82; 17.0%). The presence of NOTCH1 mutations was significantly correlated with a good prednisone response and favorable minimal residual disease (MRD) kinetics, which was independent from sex, age, white blood cell count, and T-cell immunophenotype at the time of diagnosis. Furthermore, activating NOTCH1 mutations specified a large subgroup of patients with an excellent prognosis. These findings indicate that in the context of the ALL-BFM 2000 treatment strategy, NOTCH1 mutations predict a more rapid early treatment response and a favorable long-term outcome in children with T-ALL.
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| pubmed:language |
eng
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| pubmed:journal | |
| pubmed:citationSubset |
AIM
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| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Antineoplastic Agents, Hormonal,
http://linkedlifedata.com/resource/pubmed/chemical/Asparaginase,
http://linkedlifedata.com/resource/pubmed/chemical/Daunorubicin,
http://linkedlifedata.com/resource/pubmed/chemical/NOTCH1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Prednisone,
http://linkedlifedata.com/resource/pubmed/chemical/Receptor, Notch1,
http://linkedlifedata.com/resource/pubmed/chemical/Vincristine
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| pubmed:status |
MEDLINE
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| pubmed:month |
Aug
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| pubmed:issn |
0006-4971
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| pubmed:author | |
| pubmed:issnType |
Print
|
| pubmed:day |
15
|
| pubmed:volume |
108
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
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| pubmed:pagination |
1151-7
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| pubmed:dateRevised |
2007-11-15
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| pubmed:meshHeading |
pubmed-meshheading:16614245-Adolescent,
pubmed-meshheading:16614245-Antineoplastic Agents, Hormonal,
pubmed-meshheading:16614245-Antineoplastic Combined Chemotherapy Protocols,
pubmed-meshheading:16614245-Asparaginase,
pubmed-meshheading:16614245-Child,
pubmed-meshheading:16614245-Child, Preschool,
pubmed-meshheading:16614245-Daunorubicin,
pubmed-meshheading:16614245-Dimerization,
pubmed-meshheading:16614245-Female,
pubmed-meshheading:16614245-Humans,
pubmed-meshheading:16614245-Leukocyte Count,
pubmed-meshheading:16614245-Male,
pubmed-meshheading:16614245-Mutation,
pubmed-meshheading:16614245-Neoplasm, Residual,
pubmed-meshheading:16614245-Precursor Cell Lymphoblastic Leukemia-Lymphoma,
pubmed-meshheading:16614245-Prednisone,
pubmed-meshheading:16614245-Receptor, Notch1,
pubmed-meshheading:16614245-Time Factors,
pubmed-meshheading:16614245-Treatment Outcome,
pubmed-meshheading:16614245-Vincristine
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| pubmed:year |
2006
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| pubmed:articleTitle |
Activating NOTCH1 mutations predict favorable early treatment response and long-term outcome in childhood precursor T-cell lymphoblastic leukemia.
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| pubmed:affiliation |
Department of Pediatric Oncology, Hematology and Immunology, University of Heidelberg, D-69120 Heidelberg, Germany.
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| pubmed:publicationType |
Journal Article,
Clinical Trial,
Research Support, Non-U.S. Gov't,
Multicenter Study
|