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Profile Name KMT2A rearrange
Gene Variant Detail

KMT2A rearrange (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 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 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 + 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 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 + 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 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 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 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 acute myeloid leukemia sensitive CYC065 Preclinical - Cell line xenograft Actionable In a preclinical study, CYC065 (Fadraciclib) treatment resulted in decreased cell viability in an acute myeloid leukemia cell line harboring a KMT2A rearrangement in culture and inhibited tumor growth in a cell line xenograft model (PMID: 32645016). 32645016
KMT2A rearrange acute lymphoblastic leukemia predicted - sensitive CYC065 Preclinical - Cell culture Actionable In a preclinical study, acute lymphoblastic leukemia cell lines harboring KMT2A rearrangements were sensitive to treatment with CYC065 (Fadraciclib) in culture (PMID: 32645016). 32645016
KMT2A rearrange acute leukemia predicted - sensitive Revumenib Phase I Actionable In a Phase I trial (AUGMENT 101), Revumenib treatment demonstrated acceptable safety and promising antileukemic activity in patients with acute leukemia harboring KMT2A rearrangements, and led to a composite complete response rate of 49% (17/35) (Blood (2021) 138 (Supplement 1): 699). detail...
KMT2A rearrange acute myeloid leukemia predicted - sensitive Revumenib Case Reports/Case Series Actionable In a Phase I trial (AUGMENT-101), Revumenib treatment resulted in an initial response with morphological leukemia-free states in 2 patients with acute myeloid leukemia harboring KMT2A rearrangements (PMID: 36922589; NCT04065399). 36922589
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 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 childhood B-cell acute lymphoblastic leukemia not applicable N/A Guideline Prognostic KMT2A rearrangements are associated with a poor prognosis in pediatric patients with B-cell acute lymphoblastic leukemia (NCCN.org). detail...
KMT2A rearrange B-cell acute lymphoblastic leukemia not applicable N/A Guideline Prognostic KMT2A rearrangements are associated with a poor prognosis in patients with B-cell acute lymphoblastic leukemia (NCCN.org). detail...