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Acute myeloid leukemia with 11q23 abnormalities

MedGen UID:
266234
•Concept ID:
C1292775
•
Neoplastic Process
Synonyms: acute myeloid leukaemia with 11q23 (MLL) abnormalities; acute myeloid leukaemia with MLL abnormalities; acute myeloid Leukaemia with t(9;11)(p21.3;q23.3); MLLT3-KMT2A; acute myeloid leukaemia with t(9;11)(p22.3;q23.3); MLLT3-KMT2A; acute myeloid leukaemia with t(9;11)(p22;q23); MLLT3-MLL; acute myeloid leukemia with 11q23 (MLL) abnormalities; Acute Myeloid Leukemia with 11q23 Abnormalities; acute myeloid leukemia with 11q23 abnormalities; Acute myeloid leukemia with 11q23 abnormality; Acute Myeloid Leukemia with KMT2A Rearrangement; Acute Myeloid Leukemia with KMT2A/MLL Rearrangement; acute myeloid leukemia with MLL abnormalities; Acute Myeloid Leukemia with MLL Rearrangement; Acute Myeloid Leukemia with MLLr; acute myeloid Leukemia with t(9;11)(p21.3;q23.3); MLLT3-KMT2A; acute myeloid leukemia with t(9;11)(p22.3;q23.3); MLLT3-KMT2A; acute myeloid leukemia with t(9;11)(p22;q23); MLLT3-MLL; Acute myeloid leukemia, 11q23 abnormalities; Acute myeloid leukemia, MLL; AML with 11q23 abnormalities; AML with t(9;11)(p22;q23); MLLT3-MLL
SNOMED CT: Acute myeloid leukemia, MLL (128829008); Acute myeloid leukemia, 11q23 abnormalities (128829008); Acute myeloid leukemia with 11q23 abnormality (1157157006)
 
Monarch Initiative: MONDO:0020317
Orphanet: ORPHA98831

Definition

A rare tumor arising from hematopoietic and lymphoid tissues characterized by abnormal proliferation and differentiation of a clonal population of myeloid stem cells carrying unspecific 11q23 abnormalities. Clinical manifestations result from accumulation of malignant myeloid cells within the bone marrow, peripheral blood and other organs, and include leukocytosis, anemia, thrombocytopenia, fatigue, anorexia and weight loss. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute myeloid leukemia with 11q23 abnormalities
Follow this link to review classifications for Acute myeloid leukemia with 11q23 abnormalities in Orphanet.

Professional guidelines

PubMed

Primary myelofibrosis: 2013 update on diagnosis, risk-stratification, and management.
Tefferi A
Am J Hematol 2013 Feb;88(2):141-50. doi: 10.1002/ajh.23384. PMID: 23349007
Primary myelofibrosis: 2012 update on diagnosis, risk stratification, and management.
Tefferi A
Am J Hematol 2011 Dec;86(12):1017-26. doi: 10.1002/ajh.22210. PMID: 22086865
Genetic abnormalities in leukemia secondary to treatment in patients with Hodgkin's disease.
Salas C, Pérez-Vera P, Frías S
Rev Invest Clin 2011 Jan-Feb;63(1):53-63. PMID: 21574543

Recent clinical studies

Therapy

Genetic abnormalities in leukemia secondary to treatment in patients with Hodgkin's disease.
Salas C, Pérez-Vera P, Frías S
Rev Invest Clin 2011 Jan-Feb;63(1):53-63. PMID: 21574543
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
Therapy-related acute lymphoblastic leukemia without 11q23 abnormality: report of six cases and a literature review.
Chen W, Wang E, Lu Y, Gaal KK, Huang Q
Am J Clin Pathol 2010 Jan;133(1):75-82. doi: 10.1309/AJCPYWC6AQC7BAVJ. PMID: 20023261
Clinical heterogeneity in childhood acute lymphoblastic leukemia with 11q23 rearrangements.
Pui CH, Chessells JM, Camitta B, Baruchel A, Biondi A, Boyett JM, Carroll A, Eden OB, Evans WE, Gadner H, Harbott J, Harms DO, Harrison CJ, Harrison PL, Heerema N, Janka-Schaub G, Kamps W, Masera G, Pullen J, Raimondi SC, Richards S, Riehm H, Sallan S, Sather H, Shuster J, Silverman LB, Valsecchi MG, Vilmer E, Zhou Y, Gaynon PS, Schrappe M
Leukemia 2003 Apr;17(4):700-6. doi: 10.1038/sj.leu.2402883. PMID: 12682627
Cytogenetic and molecular genetic evolution of chronic myeloid leukemia.
Johansson B, Fioretos T, Mitelman F
Acta Haematol 2002;107(2):76-94. doi: 10.1159/000046636. PMID: 11919388

Prognosis

MECOM gene overexpression in pediatric patients with acute myeloid leukemia.
Elsherif M, Hammad M, Hafez H, Yassin D, Ashraf M, Yasser N, Lehmann L, Elhaddad A
Acta Oncol 2022 Apr;61(4):516-522. Epub 2022 Jan 17 doi: 10.1080/0284186X.2022.2025611. PMID: 35038958
11q23/MLL rearrangements in adult acute leukemia.
Zotova OV, Lukianova AS, Valchuk MO, Karol YS, Shalay OO, Novak VL, Loginsky VE
Exp Oncol 2021 Sep;43(3):229-233. doi: 10.32471/exp-oncology.2312-8852.vol-43-no-3.16495. PMID: 34591430
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
Review: Aberrant EVI1 expression in acute myeloid leukaemia.
Hinai AA, Valk PJ
Br J Haematol 2016 Mar;172(6):870-8. Epub 2016 Jan 5 doi: 10.1111/bjh.13898. PMID: 26729571
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

Cytogenetic abnormalities predict survival after allogeneic hematopoietic stem cell transplantation for pediatric acute myeloid leukemia: a PDWP/EBMT study.
Sharma A, Galimard JE, Pryce A, Bhoopalan SV, Dalissier A, Dalle JH, Locatelli F, Jubert C, Mirci-Danicar O, Kitra-Roussou V, Bertrand Y, Fagioli F, Rialland F, Biffi A, Wynn RF, Michel G, Tambaro FP, Al-Ahmari A, Tbakhi A, Furness CL, Diaz MA, Sedlacek P, Bodova I, Faraci M, Rao K, Kleinschmidt K, Petit A, Gibson B, Bhatt NS, Kalwak K, Corbacioglu S
Bone Marrow Transplant 2024 Apr;59(4):451-458. Epub 2024 Jan 15 doi: 10.1038/s41409-024-02197-3. PMID: 38225386
Mutational landscape and clinical outcome of pediatric acute myeloid leukemia with 11q23/KMT2A rearrangements.
Yuen KY, Liu Y, Zhou YZ, Wang Y, Zhou DH, Fang JP, Xu LH
Cancer Med 2023 Jan;12(2):1418-1430. Epub 2022 Jul 14 doi: 10.1002/cam4.5026. PMID: 35833755Free 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
The biological and clinical significance of MLL abnormalities in haematological malignancies.
Mitterbauer-Hohendanner G, Mannhalter C
Eur J Clin Invest 2004 Aug;34 Suppl 2:12-24. doi: 10.1111/j.0960-135X.2004.01366.x. PMID: 15291802
Definition of acute biphenotypic leukemia.
Matutes E, Morilla R, Farahat N, Carbonell F, Swansbury J, Dyer M, Catovsky D
Haematologica 1997 Jan-Feb;82(1):64-6. PMID: 9107085

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