U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

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

Etiology

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
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
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

Diagnosis

Cytogenetic Characteristics of de novo Acute Myeloid Leukemia in Southern Vietnam.
Thao LTT, Ha CT, Ha NTT, Beaupha SMC, Nghia H, Tung TT, Son NT, Binh NT, Dung PC, Vu HA, Xinh PT
Asian Pac J Cancer Prev 2023 May 1;24(5):1789-1795. doi: 10.31557/APJCP.2023.24.5.1789. PMID: 37247302Free PMC Article
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
Acute Myeloid Leukemia With Recurrent Cytogenetic Abnormalities.
Foucar K, Anastasi J
Am J Clin Pathol 2015 Jul;144(1):6-18. doi: 10.1309/AJCPI9C8UILYQTNS. PMID: 26071458
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

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 myeloid leukemia with 11q23 abnormality coexisting with refractory metastatic Ewing sarcoma: report of a case and review of the literature.
Worcester HD, Vasef MA
Pediatr Dev Pathol 2010 Jan-Feb;13(1):50-4. doi: 10.2350/09-03-621-CR.1. PMID: 19925255
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
The human homologue of rat NG2, a chondroitin sulfate proteoglycan, is not expressed on the cell surface of normal hematopoietic cells but is expressed by acute myeloid leukemia blasts from poor-prognosis patients with abnormalities of chromosome band 11q23.
Smith FO, Rauch C, Williams DE, March CJ, Arthur D, Hilden J, Lampkin BC, Buckley JD, Buckley CV, Woods WG, Dinndorf PA, Sorensen P, Kersey J, Hammond D, Bernstein ID
Blood 1996 Feb 1;87(3):1123-33. PMID: 8562938

Prognosis

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
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
A novel gene, AF-1p, fused to HRX in t(1;11)(p32;q23), is not related to AF-4, AF-9 nor ENL.
Bernard OA, Mauchauffe M, Mecucci C, Van den Berghe H, Berger R
Oncogene 1994 Apr;9(4):1039-45. PMID: 8134107
Chromosome 11q23 abnormalities in leukaemia.
Ridge SA, Wiedemann LM
Leuk Lymphoma 1994 Jun;14(1-2):11-7. doi: 10.3109/10428199409049646. PMID: 7920216

Clinical prediction guides

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
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
A novel gene, AF-1p, fused to HRX in t(1;11)(p32;q23), is not related to AF-4, AF-9 nor ENL.
Bernard OA, Mauchauffe M, Mecucci C, Van den Berghe H, Berger R
Oncogene 1994 Apr;9(4):1039-45. PMID: 8134107
Chromosome 11q23 abnormalities in leukaemia.
Ridge SA, Wiedemann LM
Leuk Lymphoma 1994 Jun;14(1-2):11-7. doi: 10.3109/10428199409049646. PMID: 7920216

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...