Molecular Profile Detail

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Profile Name KMT2A - MLLT3
Gene Variant Detail

KMT2A - MLLT3 (gain of function)

Relevant Treatment Approaches

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Molecular Profile Indication/Tumor Type Response Type Relevant Treatment Approaches Therapy Name Approval Status Evidence Type Efficacy Evidence References
KMT2A - MLLT3 acute myeloid leukemia sensitive Dinaciclib Preclinical Actionable In a preclinical study, treatment with Dinaciclib resulted in induced cell death of acute myeloid leukemia cells harboring KMT2A-MLLT3 in culture (PMID: 26627013). 26627013
KMT2A - MLLT3 leukemia sensitive IRAK1/4 Preclinical Actionable In a preclinical study, a transgenic leukemic mouse model harboring KMT2A-MLLT3 demonstrated sensitivity to IRAK1/4, resulting in delayed progression and improved survival (PMID: 28065413). 28065413
KMT2A - MLLT3 acute myeloid leukemia sensitive I-BET151 + SGC0946 Preclinical - Patient cell culture Actionable In a preclinical study, I-BET151 and SGC0946 synergistically inhibited proliferation of patient-derived acute myeloid leukemia cells harboring KMT2A-MLLT3 in culture, and prolonged survival in animal models of mouse KMT2A-MLLT3-positive leukemia (PMID: 27294782). 27294782
KMT2A - MLLT3 acute myeloid leukemia sensitive MI-503 Preclinical - Patient cell culture Actionable In a preclinical study, MI-503 inhibited growth of an acute myeloid leukemia (AML) cell line and primary AML samples harboring KMT2A-MLLT3 (MLL-AF9) in culture (PMID: 25817203). 25817203
KMT2A - MLLT3 leukemia sensitive MI-503 Preclinical Actionable In a preclinical study, MI-503 reduced progression and improved survival of a mouse leukemia model expressing KMT2A-MLLT3 (MLL-AF9) (PMID: 25817203). 25817203
KMT2A - MLLT3 acute myeloid leukemia sensitive GSK3203591 + MS023 Preclinical - Cell culture Actionable In a preclinical study, MS023 and GSK3203591 synergistically inhibited survival of murine KMT2A-MLLT3 (reported as MLL-AF9)-driven acute myeloid leukemia (AML) cells and human AML cell lines harboring KMT2A-MLLT3 in culture (PMID: 31408619). 31408619
KMT2A - MLLT3 acute myeloid leukemia sensitive E7107 + MS023 Preclinical - Cell culture Actionable In a preclinical study, MS023 and E7107 synergistically inhibited survival of murine KMT2A-MLLT3 (reported as MLL-AF9)-driven acute myeloid leukemia cells in culture, regardless of SRSF2 mutation status (PMID: 31408619). 31408619
KMT2A - MLLT3 acute myeloid leukemia sensitive E7107 + GSK3203591 Preclinical - Cell culture Actionable In a preclinical study, E7107 and GSK3203591 synergistically inhibited survival of murine KMT2A-MLLT3 (reported as MLL-AF9)-driven acute myeloid leukemia cells in culture, regardless of SRSF2 mutation status (PMID: 31408619). 31408619
KMT2A - MLLT3 acute myeloid leukemia predicted - sensitive CYC065 Preclinical - Cell culture Actionable In a preclinical study, an acute myeloid leukemia cell line harboring KMT2A-MLLT3 was sensitive to treatment with CYC065 (Fadraciclib) in culture (PMID: 32645016). 32645016
KMT2A - MLLT3 acute myeloid leukemia sensitive Tretinoin Preclinical - Cell culture Actionable In a preclinical study, treatment with Vesanoid (tretinoin) in acute myeloid leukemia cells harboring a KMT2A-MLLT3 fusion induced expression of myeloid differentiation markers, CD11b and PU.1, and resulted in growth inhibition, and in a murine cell model resulted in upregulation of Mac-1 and C/EBPalpha and growth inhibition in culture (PMID: 23063977). 23063977
KMT2A - MLLT3 acute myeloid leukemia sensitive Decitabine + Tretinoin Preclinical - Cell culture Actionable In a preclinical study, combination of Vesanoid (tretinoin) and Dacogen (decitabine) in acute myeloid leukemia cells harboring KMT2A-MLLT3 resulted in significant growth inhibition, and greatly induced myeloid differentiation as indicated by CD11b and C/EBPalpha expression, and downregulation of c-Myc, and in a murine cell model, resulted in greater upregulation of C/EBPalpha and growth inhibition, and increased apoptosis compared to Tretinoin alone in culture (PMID: 23063977). 23063977
KMT2A - MLLT3 acute myeloid leukemia not applicable N/A Guideline Prognostic KMT2A-MLLT3 fusions are associated with an intermediate prognosis in patients with non-APL acute myeloid leukemia (NCCN.org). detail...