Molecular pathogenesis of core binding factor leukemia: current knowledge and future prospects
S Goyama, JC Mulloy - International journal of hematology, 2011 - Springer
S Goyama, JC Mulloy
International journal of hematology, 2011•SpringerCore binding factor (CBF) acute myeloid leukemia (AML) is the most common cytogenetic
subtype of AML, defined by the presence of t (8; 21) or inv (16)/t (16; 16). The chromosomal
aberrations create AML1-ETO and CBFβ-MYH11 fusion genes that disrupt the functions of
CBF, an essential transcription factor in hematopoiesis. Despite the relatively good outcome
of patients with CBF-AML, only approximately half of the patients are cured with current
therapy, indicating the need for improved therapeutic strategies. In this review, we …
subtype of AML, defined by the presence of t (8; 21) or inv (16)/t (16; 16). The chromosomal
aberrations create AML1-ETO and CBFβ-MYH11 fusion genes that disrupt the functions of
CBF, an essential transcription factor in hematopoiesis. Despite the relatively good outcome
of patients with CBF-AML, only approximately half of the patients are cured with current
therapy, indicating the need for improved therapeutic strategies. In this review, we …
Abstract
Core binding factor (CBF) acute myeloid leukemia (AML) is the most common cytogenetic subtype of AML, defined by the presence of t(8;21) or inv(16)/t(16;16). The chromosomal aberrations create AML1-ETO and CBFβ-MYH11 fusion genes that disrupt the functions of CBF, an essential transcription factor in hematopoiesis. Despite the relatively good outcome of patients with CBF-AML, only approximately half of the patients are cured with current therapy, indicating the need for improved therapeutic strategies. In this review, we summarize current knowledge regarding altered transcriptional regulation, aberrant signaling pathways, and cooperating genetic events in CBF leukemia, and discuss challenges ahead for translating these findings into the clinic.
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