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Acute leukaemia and myelodysplastic syndromes with chromosomal rearrangement involving 11q23 locus, but not MLL gene
  1. Wenli Zuo1,2,
  2. Sa A Wang1,
  3. Courtney DiNardo3,
  4. Mariko Yabe1,
  5. Shaoying Li1,
  6. L Jeffrey Medeiros1,
  7. Guilin Tang1
  1. 1Department of Hematopathology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
  2. 2Department of Hematology, Zhengzhou University Affiliated Cancer Hospital/Henan Cancer Hospital, Zhengzhou, Henan, China
  3. 3Department of Leukemia, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA
  1. Correspondence to Dr Guilin Tang, Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 72, Houston TX 77030-4009, USA; gtang{at}mdanderson.org

Abstract

Aims Chromosome 11q23 translocations, resulting in MLL (KMT2A) rearrangement, have been well characterised in acute myeloid leukaemia (AML) and acute lymphoblastic leukaemia (ALL). However, little is known of haematopoietic neoplasms associated with 11q23 translocation but without MLL rearrangement (11q23+/MLL−). The aim of this study is to characterise such cases with 11q23+/MLL−.

Methods and results We retrospectively searched our database for cases with haematopoietic malignancies with 11q23+/MLL−. We identified nine patients, two with AML, two with B-lymphoblastic leukaemia (B-ALL); two with T-lymphoblastic leukaemia (T-ALL), two with myelodysplastic syndrome (MDS) and one with chronic myelomonocytic leukaemia (CMML). The translocations included t(X;11)(p11.2;q23), t(2;11)(p21;q23), t(6;11)(q27;q23), t(8;9;11)(q13;q13;q23), t(11;11)(p15;q23), t(11;14)(q23;q24) and t(11;15)(q23;q14). Five of six patients with acute leukaemia had received chemotherapy and detection of 11q23 translocation occurred at time of disease relapse. Both patients with MDS and the patient with CMML had 11q23 translocation detected at time of initial diagnosis, all three patients progressed to AML after >1 year on hypomethylating agent therapy. All patients received risk-adapted therapies, including stem cell transplant in five patients. At the last follow-up, eight patients died with a median overall survival of 14 months.

Conclusions 11q23+/MLL− occurs rarely, involving different partner chromosomes and showing clinical and pathological features and disease subtypes different from those cases with MLL rearrangement. 11q23+/MLL− appears to be associated with clonal evolution/disease progression in acute leukaemia, a high risk for AML progression in MDS/CMML and a high incidence of disease relapse.

  • CYTOGENETICS
  • HAEMATOPATHOLOGY
  • LEUKAEMIA
  • MYELODYSPLASIA

https://doi.org/10.1136/jclinpath-2016-203831

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    Footnotes

    • Handling editor Mary Frances McMullin

    • Contributors GT, WZ collected the data and wrote the paper. SAW, CD, MY, SL, LJM wrote and approved the manuscript.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Ethics approval University of Texas, MD Anderson Cancer Center.

    • Provenance and peer review Not commissioned; externally peer reviewed.