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t(4;11)(q21;q23)

MedGen UID:
271879
•Concept ID:
C1515769
•
Cell or Molecular Dysfunction
Synonym: t(4;11)(q21;q23.3)

Definition

A chromosomal abnormality consisting of the translocation of 4q21 with 11q23. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVt(4;11)(q21;q23)

Professional guidelines

PubMed

MLL-Rearranged Acute Leukemia with t(4;11)(q21;q23)-Current Treatment Options. Is There a Role for CAR-T Cell Therapy?
Britten O, Ragusa D, Tosi S, Kamel YM
Cells 2019 Oct 29;8(11) doi: 10.3390/cells8111341. PMID: 31671855Free PMC Article
Treatment of infants with lymphoblastic leukaemia: results of the UK Infant Protocols 1987-1999.
Chessells JM, Harrison CJ, Watson SL, Vora AJ, Richards SM; Medical Research Council Working Party on Childhood Leukaemia
Br J Haematol 2002 May;117(2):306-14. doi: 10.1046/j.1365-2141.2002.03442.x. PMID: 11972512
Establishment of a human t(4;11) leukemia in severe combined immunodeficient mice and successful treatment using anti-CD19 (B43)-pokeweed antiviral protein immunotoxin.
Jansen B, Uckun FM, Jaszcz WB, Kersey JH
Cancer Res 1992 Jan 15;52(2):406-12. PMID: 1370213

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
Allogeneic hematopoietic stem cell transplantation for adult patients with t(4;11)(q21;q23) KMT2A/AFF1 B-cell precursor acute lymphoblastic leukemia in first complete remission: impact of pretransplant measurable residual disease (MRD) status. An analysis from the Acute Leukemia Working Party of the EBMT.
Esteve J, Giebel S, Labopin M, Czerw T, Wu D, Volin L, Socié G, Yakoub-Agha I, Maertens J, Cornelissen JJ, Pigneux A, Shimoni A, Schwerdtfeger R, Labussière-Wallet H, Russell N, Schattenberg A, Chevallier P, Koza V, Foà R, Schmid C, Peric Z, Mohty M, Nagler A
Leukemia 2021 Aug;35(8):2232-2242. Epub 2021 Feb 4 doi: 10.1038/s41375-021-01135-2. PMID: 33542481
Gene expression profiling of adult t(4;11)(q21;q23)-associated acute lymphoblastic leukemia reveals a different signature from pediatric cases.
De Braekeleer E, Douet-Guilbert N, Le Bris MJ, Basinko A, Morel F, De Braekeleer M
Anticancer Res 2012 Sep;32(9):3893-9. PMID: 22993334
Hematologic malignancies with t(4;11)(q21;q23)--a cytogenetic, morphologic, immunophenotypic and clinical study of 183 cases. European 11q23 Workshop participants.
Johansson B, Moorman AV, Haas OA, Watmore AE, Cheung KL, Swanton S, Secker-Walker LM
Leukemia 1998 May;12(5):779-87. doi: 10.1038/sj.leu.2401012. PMID: 9593281
Acute mixed-lineage leukemia t(4;11)(q21;q23) generates an MLL-AF4 fusion product.
Domer PH, Fakharzadeh SS, Chen CS, Jockel J, Johansen L, Silverman GA, Kersey JH, Korsmeyer SJ
Proc Natl Acad Sci U S A 1993 Aug 15;90(16):7884-8. doi: 10.1073/pnas.90.16.7884. PMID: 7689231Free PMC Article

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
Lymphoma- and leukemia-associated chromosomal translocations in healthy individuals.
Janz S, Potter M, Rabkin CS
Genes Chromosomes Cancer 2003 Mar;36(3):211-23. doi: 10.1002/gcc.10178. PMID: 12557221
Acute lymphoblastic leukaemia: diagnosis and classification.
Kebriaei P, Anastasi J, Larson RA
Best Pract Res Clin Haematol 2002 Dec;15(4):597-621. doi: 10.1053/beha.2002.0224. PMID: 12617866
Quantitative acute leukemia cytogenetics.
Mitelman F, Heim S
Genes Chromosomes Cancer 1992 Jul;5(1):57-66. doi: 10.1002/gcc.2870050109. PMID: 1384663
Translocation 4; 11 in acute lymphoblastic leukemia: clinical characteristics and prognostic significance.
Arthur DC, Bloomfield CD, Lindquist LL, Nesbit ME Jr
Blood 1982 Jan;59(1):96-9. PMID: 6947832

Therapy

Activity of immunoproteasome inhibitor ONX-0914 in acute lymphoblastic leukemia expressing MLL-AF4 fusion protein.
Jenkins TW, Downey-Kopyscinski SL, Fields JL, Rahme GJ, Colley WC, Israel MA, Maksimenko AV, Fiering SN, Kisselev AF
Sci Rep 2021 May 25;11(1):10883. doi: 10.1038/s41598-021-90451-9. PMID: 34035431Free PMC Article
The clinical characteristics, therapy and outcome of 85 adults with acute lymphoblastic leukemia and t(4;11)(q21;q23)/MLL-AFF1 prospectively treated in the UKALLXII/ECOG2993 trial.
Marks DI, Moorman AV, Chilton L, Paietta E, Enshaie A, DeWald G, Harrison CJ, Fielding AK, Foroni L, Goldstone AH, Litzow MR, Luger SM, McMillan AK, Racevskis J, Rowe JM, Tallman MS, Wiernik P, Lazarus HM
Haematologica 2013 Jun;98(6):945-52. Epub 2013 Jan 24 doi: 10.3324/haematol.2012.081877. PMID: 23349309Free PMC Article
Clinical significance of MLL-AF4 fusion transcript expression in the absence of a cytogenetically detectable t(4;11)(q21;q23) chromosomal translocation.
Uckun FM, Herman-Hatten K, Crotty ML, Sensel MG, Sather HN, Tuel-Ahlgren L, Sarquis MB, Bostrom B, Nachman JB, Steinherz PG, Gaynon PS, Heerema N
Blood 1998 Aug 1;92(3):810-21. PMID: 9680349
Acute lymphoid leukemia with t(4;11) (q21;q23) following chemotherapy with cytostatic agents targeting at DNA-topoisomerase II.
Pedersen-Bjergaard J
Leuk Res 1992 Aug;16(8):733-5. doi: 10.1016/0145-2126(92)90150-6. PMID: 1326684
Cytogenetic study in therapy-related myelodysplastic syndromes (t-MDS) and acute non-lymphocytic leukaemia (t-ANLL).
Sozzi G, Miozzo M, Orazi A, Calderone C, Castellano M, Viviani S, Santoro A, Pierotti MA, Della Porta G
Br J Cancer 1990 Mar;61(3):425-8. doi: 10.1038/bjc.1990.93. PMID: 2328210Free PMC Article

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
The RS4;11 cell line as a model for leukaemia with t(4;11)(q21;q23): Revised characterisation of cytogenetic features.
Ragusa D, Makarov EM, Britten O, Moralli D, Green CM, Tosi S
Cancer Rep (Hoboken) 2019 Oct;2(5):e1207. Epub 2019 Aug 7 doi: 10.1002/cnr2.1207. PMID: 32721124Free PMC Article
MLL-Rearranged Acute Leukemia with t(4;11)(q21;q23)-Current Treatment Options. Is There a Role for CAR-T Cell Therapy?
Britten O, Ragusa D, Tosi S, Kamel YM
Cells 2019 Oct 29;8(11) doi: 10.3390/cells8111341. PMID: 31671855Free PMC Article
Mixed-phenotype acute leukemia with t(4;11)(q21;q23) KMT2A-AFF1-rearranged.
Liu H
Blood 2019 Mar 14;133(11):1265. doi: 10.1182/blood-2018-12-893016. PMID: 30872272
Acute leukemias with the t(4;11)(q21;q23).
Pui CH
Leuk Lymphoma 1992 Jun;7(3):173-9. doi: 10.3109/10428199209053620. PMID: 1477645

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
Human t(4;11)(q21;q23) acute lymphoblastic leukemia in mice with severe combined immunodeficiency.
Uckun FM, Downing JR, Chelstrom LM, Gunther R, Ryan M, Simon J, Carroll AJ, Tuel-Ahlgren L, Crist WM
Blood 1994 Aug 1;84(3):859-65. PMID: 8043867
Acute mixed-lineage leukemia t(4;11)(q21;q23) generates an MLL-AF4 fusion product.
Domer PH, Fakharzadeh SS, Chen CS, Jockel J, Johansen L, Silverman GA, Kersey JH, Korsmeyer SJ
Proc Natl Acad Sci U S A 1993 Aug 15;90(16):7884-8. doi: 10.1073/pnas.90.16.7884. PMID: 7689231Free PMC Article
Secondary acute lymphoblastic leukemia with t (4;11): report on two cases and review of the literature.
Auxenfants E, Morel P, Lai JL, Sartiaux C, Detourmignies L, Bauters F, Fenaux P
Ann Hematol 1992 Sep;65(3):143-6. doi: 10.1007/BF01695815. PMID: 1391125
Quantitative acute leukemia cytogenetics.
Mitelman F, Heim S
Genes Chromosomes Cancer 1992 Jul;5(1):57-66. doi: 10.1002/gcc.2870050109. PMID: 1384663

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