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Profile Name KMT2A R3965W
Gene Variant Detail

KMT2A R3965W (unknown)

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 - AFF1 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-AFF1 in culture (PMID: 26627013). 26627013
KMT2A - AFF1 leukemia sensitive IRAK1/4 Preclinical - Cell culture Actionable In a preclinical study, leukemia cells harboring KMT2A-AFF1 demonstrated sensitivity to IRAK1/4 in culture, resulting in decreased cell proliferation (PMID: 28065413). 28065413
KMT2A - AFF1 B-cell acute lymphoblastic leukemia predicted - resistant Blinatumomab Case Reports/Case Series Actionable In a clinical case study, Blincyto (blinatumomab) treatment in a patient with B-lymphoblastic leukemia harboring KMT2A-AFF1 resulted in lineage switch as demonstrated by identification of myeloid sarcoma of the breast and acute myeloid leukemia within one month of treatment onset, along with mixed phenotype acute leukemia observed following cessation of treatment (PMID: 31885955). 31885955
KMT2A - AFF1 acute myeloid leukemia predicted - sensitive AZD5153 Preclinical - Cell line xenograft Actionable In a preclinical study, AZD5153 inhibited proliferation of acute myeloid leukemia cells harboring KMT2A-AFF1 (MLL-AF4) in culture, resulted in tumor growth inhibition in cell line xenograft models (PMID: 27573426). 27573426
KMT2A - AFF1 leukemia sensitive I-BET151 + SGC0946 Preclinical - Cell culture Actionable In a preclinical study, I-BET151 and SGC0946 synergistically inhibited proliferation of leukemia cells harboring KMT2A-AFF1 in culture (PMID: 27294782). 27294782
KMT2A - AFF1 acute lymphoblastic leukemia predicted - sensitive AZD5153 Preclinical - Cell line xenograft Actionable In a preclinical study, AZD5153 inhibited proliferation of acute lymphocytic leukemia cells harboring KMT2A-AFF1 (MLL-AF4) in culture, resulted in tumor growth inhibition in cell line xenograft models (PMID: 27573426). 27573426
KMT2A - AFF1 acute myeloid leukemia sensitive MI-503 Preclinical - Cell line xenograft Actionable In a preclinical study, MI-503 reduced expression of KMT2A (MLL) fusion target genes, inhibited growth of an acute myeloid leukemia (AML) cell lines harboring KMT2A-AFF1 (MLL-AF4) in culture, and inhibited tumor growth and progression in an AML cell line xenograft model harboring KMT2A-AFF1 (MLL-AF4) (PMID: 25817203). 25817203
KMT2A fusion acute myeloid leukemia predicted - sensitive MI-503 Preclinical - Cell culture Actionable In a preclinical study, MI-503 inhibited growth of several acute myeloid leukemia (AML) cell lines and primary AML samples harboring KMT2A (MLL) fusions in culture (PMID: 25817203). 25817203
KMT2A fusion acute myeloid leukemia sensitive CDKI-73 Preclinical - Cell line xenograft Actionable In a preclinical study, CDKI-73 treatment inhibited Cdk9 kinase activity and viability, and induced apoptosis in acute myeloid leukemia cell lines harboring KMT2A fusions in culture, and inhibited tumor growth and induced regression in a cell line xenograft model (PMID: 30194564). 30194564
KMT2A fusion 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 fusions in culture (PMID: 27294782). 27294782
KMT2A - MLLT1 acute myeloid leukemia sensitive MI-503 Preclinical - Cell culture Actionable In a preclinical study, MI-503 inhibited growth of an acute myeloid leukemia cell line harboring KMT2A-MLLT1 (MLL-ENL) in culture (PMID: 25817203). 25817203
KMT2A - MLLT1 NRAS G12D acute myeloid leukemia predicted - sensitive AZD0156 Preclinical Actionable In a preclinical study, AZD0156 treatment resulted in growth inhibition of tumor cells and prolonged survival in animal models of acute myeloid leukemia harboring KMT2A-MLLT1 fusion and NRAS G12D (PMID: 27625305). 27625305
KMT2A - MLLT1 NRAS G12D acute myeloid leukemia predicted - sensitive AZ20 Preclinical Actionable In a preclinical study, AZ20 treatment resulted in growth inhibition of tumor cells and prolonged survival in animal models of acute myeloid leukemia harboring KMT2A-MLLT1 fusion and NRAS G12D (PMID: 27625305). 27625305
KMT2A - MLLT1 NRAS G12D acute myeloid leukemia sensitive Dinaciclib Preclinical Actionable In a preclinical study, acute myeloid leukemia cells co-harboring KMT2A-MLLT1 and NRAS G12D demonstrated a decrease in tumor burden, reduced colony formation, and an increase in cell death when treated with Dinaciclib in both culture and mouse models (PMID: 26627013). 26627013
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Romidepsin Preclinical - Cell line xenograft Actionable In a preclinical study, Istodax (romidepsin) enhanced the effects of Cytosar-U (cytarabine) in acute lymphocytic leukemia cell lines harboring a KMT2A rearrangement in culture, and in cell line xenograft models, demonstrating decreased leukemic cells by 73% (PMID: 27443263). 27443263
KMT2A rearrange acute lymphoblastic leukemia decreased response Romidepsin Preclinical - Cell line xenograft Actionable In a preclinical study, Istodax (romidepsin) resulted in minimal activity in acute lymphocytic leukemia cell lines harboring a KMT2A rearrangement in culture, and in cell line xenograft models, demonstrating decreased leukemic cells by 16% (PMID: 27443263). 27443263
KMT2A rearrange acute leukemia predicted - sensitive Pinometostat Case Reports/Case Series Actionable In a Phase I trial, Pinometostat (EPZ-5676) treatment resulted in complete remission in 2 patients with KMT2A rearranged (both with t(11;19)) acute leukemia (PMID: 29724899; NCT01684150). 29724899
KMT2A rearrange leukemia predicted - sensitive I-CBP112 + JQ1 Preclinical Actionable In a preclinical study, I-CBP112 sensitized leukemia cells harboring KMT2A fusions to JQ1, resulting in decreased cell growth in culture (PMID: 26552700). 26552700
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Panobinostat Preclinical - Cell culture Actionable In a preclinical study, Farydak (panobinostat) enhanced the effects of Cytosar-U (cytarabine) in acute lymphoblastic leukemia cells harboring a KMT2A rearrangement in culture, resulting in cell death (PMID: 27443263). 27443263
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Dacinostat Preclinical - Cell culture Actionable In a preclinical study, Dacinostat (LAQ824) enhanced the effects of Cytosar-U (cytarabine) in acute lymphoblastic leukemia cell lines harboring a KMT2A rearrangement in culture, resulting in cell death (PMID: 27443263). 27443263
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine Preclinical - Cell line xenograft Actionable In a preclinical study, Cytosar-U (cytarabine) decreased leukemic cells by 66% in acute lymphocytic leukemia cell line xenograft models harboring a KMT2A rearrangement (PMID: 27443263). 27443263
KMT2A rearrange acute myeloid leukemia not applicable N/A Guideline Prognostic KMT2A rearrangements (t(v;11q23.3)) are associated with a poor/adverse prognosis in patients with non-APL acute myeloid leukemia (NCCN.org). detail...
KMT2A rearrange acute myeloid leukemia not applicable N/A Clinical Study Prognostic In multiple clinical studies, KMT2A rearrangements, specifically partial tandem duplications, were associated with a poor overall survival in acute myeloid leukemia patients (PMID: 24487413, PMID: 22915647, PMID: 22417203, PMID: 21881046). 21881046 24487413 22417203 22915647
KMT2A rearrange leukemia predicted - sensitive Doxorubicin + I-CBP112 Preclinical Actionable In a preclinical study, I-CBP112 sensitized leukemia cells harboring KMT2A fusions to Doxorubicin, leading to decreased cell growth in culture (PMID: 26552700). 26552700
KMT2A rearrange acute lymphoblastic leukemia sensitive Cytarabine + Mocetinostat Preclinical - Cell culture Actionable In a preclinical study, Mocetinostat (MGCD0103) enhanced the effects of Cytosar-U (cytarabine) in acute lymphoblastic leukemia cell lines harboring a KMT2A rearrangement in culture, resulting in cell death (PMID: 27443263). 27443263
KMT2A rearrange acute myeloid leukemia predicted - sensitive KO-539 Preclinical - Cell line xenograft Actionable In a preclinical study, KO-539 inhibited growth of KMT2A (MLL1)-rearranged acute myeloid leukemia cells in culture, and prolonged survival in cell-line xenograft models (AACR; Mol Cancer Ther 2018;17(1 Suppl):Abstract nr LB-A27). detail...
KMT2A - EP300 acute myeloid leukemia sensitive MI-503 Preclinical - Patient cell culture Actionable In a preclinical study, MI-503 inhibited growth of a primary acute myeloid sample harboring KMT2A-EP300 in culture (PMID: 25817203). 25817203
KMT2A - ELL 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-ELL in culture (PMID: 27294782). 27294782
KMT2A - MLLT10 leukemia sensitive EPZ004777 Preclinical Actionable In a preclinical study, transformed cells expressing the fusion, KMT2A-MLLT10, demonstrated sensitivity to EPZ004777 in culture and in mouse models, resulting in cell cycle arrest, increased apoptotic activity, and inhibition of cell proliferation (PMID: 23138183). 23138183
KMT2A - MLLT10 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-MLLT10 in culture (PMID: 27294782). 27294782
KMT2A - GAS7 leukemia sensitive AMI-408 Preclinical - Cell line xenograft Actionable In a preclinical study, treatment with AMI-408 decreased growth of leukemia cells expressing KMT2A-GAS7 (MLL-GAS7) in culture, and increased survival in xenograft models (PMID: 26766589). 26766589
KMT2A - SEPTIN6 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-SEPTIN6 in culture (PMID: 27294782). 27294782
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 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 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 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 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 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...
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 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 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 SRSF2 P95H acute myeloid leukemia sensitive EPZ015666 Preclinical Actionable In a preclinical study, EPZ015666 (GSK3235025) treatment decreased SDMA levels and prolonged survival in a mouse model of KMT2A-MLLT3 (MLL-AF9)-driven acute myeloid leukemia harboring SRSF2 P95H, but not in a SRSR2 wild-type model (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive MS023 Preclinical Actionable In a preclinical study, MS023 inhibited survival of murine KMT2A-MLLT3 ( reported as MLL-AF9)-driven acute myeloid leukemia cells harboring SRSF2 P95H in culture and delayed disease progression in mouse models, but not in wild-type SRSF2 models (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive H3B-8800 Preclinical Actionable In a preclinical study, H3B-8800 inhibited tumor growth in transplant animal models of mouse acute myeloid leukemia cells harboring SRSF2 P95H and KMT2A-MLLT3 (MLL-AF9) while had no effect in SRSF2 wild-type controls (Blood 2016 128:966). detail...
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive GSK3203591 Preclinical - Cell culture Actionable In a preclinical study, murine KMT2A-MLLT3 ( reported as MLL-AF9)-driven acute myeloid leukemia cells expressing SRSF2 P95H demonstrated increased sensitivity to GSK3203591 compared to cells expressing wild-type SRSF2 in culture (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive E7107 Preclinical - Cell culture Actionable In a preclinical study, murine KMT2A-MLLT3 ( reported as MLL-AF9)-driven acute myeloid leukemia cells expressing SRSF2 P95H demonstrated increased sensitivity to E7107 compared to cells expressing wild-type SRSF2 in culture (PMID: 31408619). 31408619
KMT2A - MLLT3 SRSF2 P95H acute myeloid leukemia sensitive E7107 Preclinical Actionable In a preclinical study, E7107 treatment decreased tumor burden and prolonged survival in an isogenic mouse model of acute myeloid leukemia expressing KMT2A-MLLT3 (reported as MLL-AF9) and SRSF2 P95H, but not in a model expressing KMT2A-MLLT3 and wild-type SRSF2 (PMID: 27135740). 27135740
Clinical Trial Phase Therapies Title Recruitment Status