Childhood core binding factor (CBF) acute myeloid leukemia and its association with French American British (FAB) classification

2020 ◽  
pp. 1-11
Author(s):  
Naeem Jabbar ◽  
Neelum Mansoor ◽  
Sidra Maqsood ◽  
Zaid Muhammad Aslam ◽  
Anzal Taufeeq Jangda ◽  
...  
Keyword(s):  
Acute Myeloid Leukaemia ◽  
Myeloid Leukaemia ◽  
White Cell Count ◽  
Core Binding Factor ◽  
Mean Values ◽  
Binding Factor ◽  
Significant Difference ◽  
Cytogenetic Testing ◽  
The Difference ◽  
Acute Myeloid

Abstract Objective: To find the frequency of core binding factor acute myeloid leukaemia in our population, and to determine its association with morphological subtypes. Methods: The retrospective study was conducted at The Indus Hospital, Karachi, and comprised data of patients aged 1-17 years who were diagnosed with acute myeloid leukaemia from July 2013 to June 2017. Data was analysed using SPSS 21. Results: Of the 237 patients, 137(58%) were males and 100(42%) were females. The overall mean age was 8±4.34 years. Cytogenetic testing had been performed in 212(89.45%) cases, and core binding factor was detected in 72(34%) cases. There was significant difference between the mean values of white cell count and the subtypes (p=0.000). Also the difference between core binding factor and the subtypes was significant (p=0.000). Conclusion: There was found to be a significant association of core binging factor with specific subgroups of acute myeloid leukaemia. Key Words: Acute myeloid leukaemia, Core binding factor, Cytogenetic abnormalities, Prognosis. Continuous....

Involvement of a common progenitor cell in core binding factor acute myeloid leukaemia associated with mastocytosis

2012 ◽  
Vol 36 (11) ◽  
pp. 1330-1333 ◽  
Author(s):  
Edouard Cornet ◽  
Florent Dumézy ◽  
Christophe Roumier ◽  
Pascale Lepelley ◽  
Nathalie Jouy ◽  
...  
Keyword(s):  
Acute Myeloid Leukaemia ◽  
Myeloid Leukaemia ◽  
Progenitor Cell ◽  
Core Binding Factor ◽  
Binding Factor ◽  
Acute Myeloid

scholarly journals Core binding factor acute myeloid leukaemia and c-KIT mutations

2013 ◽  
Vol 29 (5) ◽  
pp. 1867-1872 ◽  
Author(s):  
LUDOVICA RIERA ◽  
FILIPPO MARMONT ◽  
DANIELA TOPPINO ◽  
CHIARA FRAIRIA ◽  
FRANCESCA SISMONDI ◽  
...  
Keyword(s):  
Acute Myeloid Leukaemia ◽  
Myeloid Leukaemia ◽  
Core Binding Factor ◽  
Binding Factor ◽  
Acute Myeloid

scholarly journals Core-binding factor-β positive acute myeloid leukaemia cells induce T-cell responses

2003 ◽  
Vol 123 (5) ◽  
pp. 819-829 ◽  
Author(s):  
G.-A. Banat ◽  
Kerstin Ihlow ◽  
Nurguel Usluoglu ◽  
Sabine Hoppmann ◽  
Marianne Hoeck ◽  
...  
Keyword(s):  
T Cell ◽  
Acute Myeloid Leukaemia ◽  
Myeloid Leukaemia ◽  
T Cell Responses ◽  
Core Binding Factor ◽  
Cell Responses ◽  
Binding Factor ◽  
Acute Myeloid

The clinical spectrum of adult acute myeloid leukaemia associated with core binding factor translocations

2006 ◽  
Vol 135 (2) ◽  
pp. 165-173 ◽  
Author(s):  
Frederick R. Appelbaum ◽  
Kenneth J. Kopecky ◽  
Martin S. Tallman ◽  
Marilyn L. Slovak ◽  
Holly M. Gundacker ◽  
...  
Keyword(s):  
Acute Myeloid Leukaemia ◽  
Myeloid Leukaemia ◽  
Clinical Spectrum ◽  
Core Binding Factor ◽  
Binding Factor ◽  
Acute Myeloid

KIT Exon 8 Mutations Associated with Core Binding Factor (CBF) - Acute Myeloid Leukemia (AML) Cause Hypersensitivity of KIT to Its Natural Ligand Stem Cell Factor.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 92-92
Author(s):  
Tobias M. Kohl ◽  
Susanne Schnittger ◽  
Wolfgang Hiddemann ◽  
Karsten Spiekermann
Keyword(s):  
Acute Myeloid Leukemia ◽  
Stem Cell ◽  
Stem Cell Factor ◽  
Myeloid Leukemia ◽  
Biological Effects ◽  
Nucleotide Sequencing ◽  
Core Binding Factor ◽  
Binding Factor ◽  
Significant Difference ◽  
Acute Myeloid

Abstract Mutations in the extracellular portion of the KIT receptor tyrosine kinase (exon 8 mutations) are strongly associated with core binding factor (CBF) - acute myeloid leukemia (AML), but the functional role of these mutations has not been elucidated. In 93% of cases, codon Asp419 is deleted and exon 8 mutations were reported to confer an impaired prognosis to patients with CBF-AML. In this study, we are the first to report pro-proliferative and antiapoptotic potential of representative KIT exon 8 mutations in a cell culture model and to show a significant difference to KIT wildtype (KIT-WT). Three representative exon 8 mutants including a single deletion of codon 419 were created by in vitro site-directed mutagenesis. The integrity of all constructs was assessed by complete nucleotide sequencing. After stable expression in IL-3 dependent Ba/F3 cells (confirmed by FACS analysis and immunoblotting), exon 8 KIT mutants were characterized by a hypersensitivity to stem cell factor (SCF) stimulation in terms of proliferation and resistance to apoptotic cell death. The differences to KIT-WT occurred in the physiological range of SCF from 1 to 10ng/ml. The proliferative response caused by stimulation with SCF was reversed in KIT-WT and exon 8 mutants in the presence of Imatinib® (Novartis) in contrast to the activation loop mutant D816V which could not be inhibited. These biological effects were confirmed by demonstrating increased phosphorylation of the KIT downstream targets mitogen-activated protein kinase (MAPK) and AKT after SCF stimulation compared to the KIT-WT receptor. Furthermore, the MEK inhibitor PD98059 and the PI3 kinase inhibitor LY294002 resulted in a dose dependent inhibition of SCF induced proliferation in exon 8 mutants. Our data show that KIT exon 8 mutations represent gain-of-function mutations by inducing receptor hypersensitivity to its ligand SCF by activation of MAPK and PI3K and might represent a new molecular target for treatment of CBF leukemias.

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