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|Synonyms||IMD75 | KIAA1546 | MDS|
|Gene Description||TET2, methylcytosine dioxygenase TET2, is a member of the ten-eleven-translocation family of genes that plays a role in DNA methylation and is involved in hematopoesis (PMID: 26099018, PMID: 25510268, PMID: 27848178). TET2 has been identified as a tumor suppressor in hematological malignancies, and mutations in TET2 are common in hematological cancers including myelodysplastic syndrome, chronic myelomoncytic leukemia, and atypical CML (PMID: 26099018, PMID: 22240200, PMID: 30138727) and epigenetic dysregulation has been observed in glioblastoma (PMID: 29899831).|
|Molecular Profile||Indication/Tumor Type||Response Type||Therapy Name||Approval Status||Evidence Type||Efficacy Evidence||References|
|TET2 T1063Nfs*5 TET2 E1207K||angioimmunoblastic T-cell lymphoma||predicted - sensitive||Dasatinib||Case Reports/Case Series||Actionable||In a Phase I trial, four patients with relapsed/refractory angioimmunoblastic T-cell lymphoma harboring TET2 mutations and/or RHOA G17V achieved a partial response within 30 days of Sprycel (dasatinib) treatment, including one patient harboring TET2 T1063Nfs*5 and TET2 E1207K who maintained the partial response at the time of trial discontinuation (PMID: 32107212).||32107212|
|TET2 mutant||acute myeloid leukemia||predicted - sensitive||Azacitidine||Phase I||Actionable||In a retrospective analysis, presence of a TET2 mutation correlated with higher overall response rate (ORR) in patients with myelodysplastic syndrome and acute myeloid leukemia following treatment with Vidaza (azacitidine), with an ORR of 85% (11/3) in TET2-mutated patients, compared to 47% (34/47) in patients with wild-type TET2, but did not correlate with overall survival (PMID: 21494260).||21494260|
|TET2 mutant||acute myeloid leukemia||not applicable||N/A||Clinical Study||Prognostic||In multiple clinical studies, including a meta-analysis, TET2 mutations were associated with shorter overall survival in patients with acute myeloid leukemia (PMID: 24524305, PMID: 25412851, PMID: 24994606).||24524305 25412851 24994606|
|TET2 mutant||angioimmunoblastic T-cell lymphoma||not applicable||N/A||Guideline||Diagnostic||TET2 mutations aid in the diagnosis of angioimmunoblastic T-cell lymphoma (NCCN.org).||detail...|
|TET2 mutant||myelofibrosis||not applicable||N/A||Guideline||Diagnostic||TET2 mutations aid in the diagnosis of primary myelofibrosis in the absence of JAK2, CALR, or MPL mutations (NCCN.org).||detail...|