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t(6;11)(q27;q23)

MedGen UID:
273753
•Concept ID:
C1515779
•
Cell or Molecular Dysfunction
Synonym: t(6;11)(q27;q23.3)

Definition

A cytogenetic abnormality that refers to the translocation of the long arm (q27) of chromosome 6 and the long arm (q23) of chromosome 11. It is associated with the development of de novo acute myeloid leukemia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVt(6;11)(q27;q23)

Professional guidelines

PubMed

Treatment of adult AML with t(6;11)(q27;q23) by allogeneic hematopoietic SCT in the first CR.
Tamai H, Yamaguchi H, Takahashi S, Tojo A, Hamaguchi H, Kobayashi T, Akiyama H, Sakamaki H, Okumura H, Nakao S, Arai A, Miura O, Tajika K, Inokuchi K, Dan K
Bone Marrow Transplant 2008 Oct;42(8):553-4. Epub 2008 Jul 14 doi: 10.1038/bmt.2008.200. PMID: 18622415

Recent clinical studies

Etiology

Outcomes of Childhood Noninfant Acute Lymphoblastic Leukemia With 11q23/KMT2A Rearrangements in a Modern Therapy Era: A Retrospective International Study.
Attarbaschi A, Möricke A, Harrison CJ, Mann G, Baruchel A, De Moerloose B, Conter V, Devidas M, Elitzur S, Escherich G, Hunger SP, Horibe K, Manabe A, Loh ML, Pieters R, Schmiegelow K, Silverman LB, Stary J, Vora A, Pui CH, Schrappe M, Zimmermann M; Ponte-di-Legno Childhood Acute Lymphoblastic Leukemia Working Group
J Clin Oncol 2023 Mar 1;41(7):1404-1422. Epub 2022 Oct 18 doi: 10.1200/JCO.22.01297. PMID: 36256911Free PMC Article
Mutational landscape and clinical outcome of patients with de novo acute myeloid leukemia and rearrangements involving 11q23/KMT2A.
Bill M, Mrózek K, Kohlschmidt J, Eisfeld AK, Walker CJ, Nicolet D, Papaioannou D, Blachly JS, Orwick S, Carroll AJ, Kolitz JE, Powell BL, Stone RM, de la Chapelle A, Byrd JC, Bloomfield CD
Proc Natl Acad Sci U S A 2020 Oct 20;117(42):26340-26346. Epub 2020 Oct 5 doi: 10.1073/pnas.2014732117. PMID: 33020282Free PMC Article
Thioridazine requires calcium influx to induce MLL-AF6-rearranged AML cell death.
Tregnago C, Da Ros A, Porcù E, Benetton M, Simonato M, Simula L, Borella G, Polato K, Minuzzo S, Borile G, Cogo P, Campello S, Massi A, Romagnoli R, Buldini B, Locatelli F, Pigazzi M
Blood Adv 2020 Sep 22;4(18):4417-4429. doi: 10.1182/bloodadvances.2020002001. PMID: 32931582Free PMC Article
Refinement of cytogenetic classification in acute myeloid leukemia: determination of prognostic significance of rare recurring chromosomal abnormalities among 5876 younger adult patients treated in the United Kingdom Medical Research Council trials.
Grimwade D, Hills RK, Moorman AV, Walker H, Chatters S, Goldstone AH, Wheatley K, Harrison CJ, Burnett AK; National Cancer Research Institute Adult Leukaemia Working Group
Blood 2010 Jul 22;116(3):354-65. Epub 2010 Apr 12 doi: 10.1182/blood-2009-11-254441. PMID: 20385793
Adult de novo acute myeloid leukemia with t(6;11)(q27;q23): results from Cancer and Leukemia Group B Study 8461 and review of the literature.
Blum W, Mrózek K, Ruppert AS, Carroll AJ, Rao KW, Pettenati MJ, Anastasi J, Larson RA, Bloomfield CD
Cancer 2004 Sep 15;101(6):1420-7. doi: 10.1002/cncr.20489. PMID: 15368330

Diagnosis

Outcomes of Childhood Noninfant Acute Lymphoblastic Leukemia With 11q23/KMT2A Rearrangements in a Modern Therapy Era: A Retrospective International Study.
Attarbaschi A, Möricke A, Harrison CJ, Mann G, Baruchel A, De Moerloose B, Conter V, Devidas M, Elitzur S, Escherich G, Hunger SP, Horibe K, Manabe A, Loh ML, Pieters R, Schmiegelow K, Silverman LB, Stary J, Vora A, Pui CH, Schrappe M, Zimmermann M; Ponte-di-Legno Childhood Acute Lymphoblastic Leukemia Working Group
J Clin Oncol 2023 Mar 1;41(7):1404-1422. Epub 2022 Oct 18 doi: 10.1200/JCO.22.01297. PMID: 36256911Free PMC Article
Comprehensive immunophenotypic study of acute myeloid leukemia with KMT2A (MLL) rearrangement in adults: A single-institution experience.
Konoplev S, Wang X, Tang G, Li S, Wang W, Xu J, Pierce SA, Jia F, Jorgensen JL, Ravandi F, Issa GC, Medeiros LJ, Wang SA
Cytometry B Clin Cytom 2022 Mar;102(2):123-133. Epub 2021 Dec 28 doi: 10.1002/cyto.b.22051. PMID: 34964255
Refinement of cytogenetic classification in acute myeloid leukemia: determination of prognostic significance of rare recurring chromosomal abnormalities among 5876 younger adult patients treated in the United Kingdom Medical Research Council trials.
Grimwade D, Hills RK, Moorman AV, Walker H, Chatters S, Goldstone AH, Wheatley K, Harrison CJ, Burnett AK; National Cancer Research Institute Adult Leukaemia Working Group
Blood 2010 Jul 22;116(3):354-65. Epub 2010 Apr 12 doi: 10.1182/blood-2009-11-254441. PMID: 20385793
Adult de novo acute myeloid leukemia with t(6;11)(q27;q23): results from Cancer and Leukemia Group B Study 8461 and review of the literature.
Blum W, Mrózek K, Ruppert AS, Carroll AJ, Rao KW, Pettenati MJ, Anastasi J, Larson RA, Bloomfield CD
Cancer 2004 Sep 15;101(6):1420-7. doi: 10.1002/cncr.20489. PMID: 15368330
Immunocytochemical identification of nuclear estrogen-receptors in human acute myeloid leukemia.
Hatfill SJ, Brusnicky J, Fester E
Leuk Res 1991;15(5):315-20. doi: 10.1016/0145-2126(91)90006-f. PMID: 2046384

Therapy

Therapy-related Acute Myeloid Leukemia after the Long-term Administration of Low-dose Etoposide for Chronic-type Adult T-cell Leukemia-lymphoma: A Case Report and Literature Review.
Shimada N, Ohno N, Tanosaki R, Fuji S, Suzuki Y, Yuji K, Uchimaru K, Tojo A
Intern Med 2017;56(14):1879-1884. Epub 2017 Jul 15 doi: 10.2169/internalmedicine.56.7763. PMID: 28717086Free PMC Article
Refinement of cytogenetic classification in acute myeloid leukemia: determination of prognostic significance of rare recurring chromosomal abnormalities among 5876 younger adult patients treated in the United Kingdom Medical Research Council trials.
Grimwade D, Hills RK, Moorman AV, Walker H, Chatters S, Goldstone AH, Wheatley K, Harrison CJ, Burnett AK; National Cancer Research Institute Adult Leukaemia Working Group
Blood 2010 Jul 22;116(3):354-65. Epub 2010 Apr 12 doi: 10.1182/blood-2009-11-254441. PMID: 20385793
Distinct MLL gene rearrangements associated with successive acute monocytic and lymphoblastic leukemias in the same patient.
Jonveaux P, Hillion J, Bernard O, Le Coniat M, Derré J, Flexor M, Larsen CJ, Berger R
Leukemia 1994 Dec;8(12):2224-7. PMID: 7808011

Prognosis

Outcomes of Childhood Noninfant Acute Lymphoblastic Leukemia With 11q23/KMT2A Rearrangements in a Modern Therapy Era: A Retrospective International Study.
Attarbaschi A, Möricke A, Harrison CJ, Mann G, Baruchel A, De Moerloose B, Conter V, Devidas M, Elitzur S, Escherich G, Hunger SP, Horibe K, Manabe A, Loh ML, Pieters R, Schmiegelow K, Silverman LB, Stary J, Vora A, Pui CH, Schrappe M, Zimmermann M; Ponte-di-Legno Childhood Acute Lymphoblastic Leukemia Working Group
J Clin Oncol 2023 Mar 1;41(7):1404-1422. Epub 2022 Oct 18 doi: 10.1200/JCO.22.01297. PMID: 36256911Free PMC Article
Association of race and ethnicity with clinical phenotype, genetics, and survival in pediatric acute myeloid leukemia.
Conneely SE, McAtee CL, Gupta R, Lubega J, Scheurer ME, Rau RE
Blood Adv 2021 Dec 14;5(23):4992-5001. doi: 10.1182/bloodadvances.2021004735. PMID: 34619758Free PMC Article
Mutational landscape and clinical outcome of patients with de novo acute myeloid leukemia and rearrangements involving 11q23/KMT2A.
Bill M, Mrózek K, Kohlschmidt J, Eisfeld AK, Walker CJ, Nicolet D, Papaioannou D, Blachly JS, Orwick S, Carroll AJ, Kolitz JE, Powell BL, Stone RM, de la Chapelle A, Byrd JC, Bloomfield CD
Proc Natl Acad Sci U S A 2020 Oct 20;117(42):26340-26346. Epub 2020 Oct 5 doi: 10.1073/pnas.2014732117. PMID: 33020282Free PMC Article
Lineage switch with t(6;11)(q27;q23) from T-cell lymphoblastic lymphoma to acute monoblastic leukemia at relapse.
Higuchi Y, Tokunaga K, Watanabe Y, Kawakita T, Harada N, Yamaguchi S, Nosaka K, Mitsuya H, Asou N
Cancer Genet 2016 Jun;209(6):267-71. Epub 2016 May 18 doi: 10.1016/j.cancergen.2016.05.070. PMID: 27268298
Refinement of cytogenetic classification in acute myeloid leukemia: determination of prognostic significance of rare recurring chromosomal abnormalities among 5876 younger adult patients treated in the United Kingdom Medical Research Council trials.
Grimwade D, Hills RK, Moorman AV, Walker H, Chatters S, Goldstone AH, Wheatley K, Harrison CJ, Burnett AK; National Cancer Research Institute Adult Leukaemia Working Group
Blood 2010 Jul 22;116(3):354-65. Epub 2010 Apr 12 doi: 10.1182/blood-2009-11-254441. PMID: 20385793

Clinical prediction guides

Outcomes of Childhood Noninfant Acute Lymphoblastic Leukemia With 11q23/KMT2A Rearrangements in a Modern Therapy Era: A Retrospective International Study.
Attarbaschi A, Möricke A, Harrison CJ, Mann G, Baruchel A, De Moerloose B, Conter V, Devidas M, Elitzur S, Escherich G, Hunger SP, Horibe K, Manabe A, Loh ML, Pieters R, Schmiegelow K, Silverman LB, Stary J, Vora A, Pui CH, Schrappe M, Zimmermann M; Ponte-di-Legno Childhood Acute Lymphoblastic Leukemia Working Group
J Clin Oncol 2023 Mar 1;41(7):1404-1422. Epub 2022 Oct 18 doi: 10.1200/JCO.22.01297. PMID: 36256911Free PMC Article
The role of reciprocal fusions in MLL-r acute leukemia: studying the chromosomal translocation t(6;11).
Kundu A, Kowarz E, Marschalek R
Oncogene 2021 Oct;40(40):5902-5912. Epub 2021 Aug 5 doi: 10.1038/s41388-021-01983-3. PMID: 34354240Free PMC Article
Refinement of cytogenetic classification in acute myeloid leukemia: determination of prognostic significance of rare recurring chromosomal abnormalities among 5876 younger adult patients treated in the United Kingdom Medical Research Council trials.
Grimwade D, Hills RK, Moorman AV, Walker H, Chatters S, Goldstone AH, Wheatley K, Harrison CJ, Burnett AK; National Cancer Research Institute Adult Leukaemia Working Group
Blood 2010 Jul 22;116(3):354-65. Epub 2010 Apr 12 doi: 10.1182/blood-2009-11-254441. PMID: 20385793
Complex and cryptic chromosomal rearrangements involving the MLL gene in acute leukemia: a study of 7 patients and review of the literature.
De Braekeleer E, Meyer C, Douet-Guilbert N, Morel F, Le Bris MJ, Berthou C, Arnaud B, Marschalek R, Férec C, De Braekeleer M
Blood Cells Mol Dis 2010 Apr 15;44(4):268-74. Epub 2010 Mar 4 doi: 10.1016/j.bcmd.2010.02.011. PMID: 20206559
Analysis of the t(6;11)(q27;q23) in leukemia shows a consistent breakpoint in AF6 in three patients and in the ML-2 cell line.
Tanabe S, Zeleznik-Le NJ, Kobayashi H, Vignon C, Espinosa R 3rd, LeBeau MM, Thirman MJ, Rowley JD
Genes Chromosomes Cancer 1996 Apr;15(4):206-16. doi: 10.1002/(SICI)1098-2264(199604)15:4<206::AID-GCC2>3.0.CO;2-5. PMID: 8703846

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