MEF2D-rearranged acute lymphoblastic leukemia resembles Burkitt lymphoma/leukemia

  • Jiewen Sun
  • Wenjuan YuEmail author
  • Xiang ZhangEmail author
Letter to the Editor

Dear Editor,

Burkitt lymphoma/leukemia (BL) is one clinically aggressive hematological malignancy, and it is characterized by the morphology of monotonous medium-sized cells containing abundant basophilic cytoplasm and lipid vacuoles, as well as round nuclei with multiple nucleoli and clumped chromatin, the immunophenotype of mature B cell, and MYC rearrangement leading to its overexpression [1]. Interestingly, some acute lymphoblastic leukemia (ALL) could resemble BL in clinic, but the mechanism remained unknown. MEF2D rearrangement is frequent in B-cell ALL, and its targets mainly involved BCL9, CSF1R, DAZAP1, HNRNPUL1, SS18, STAT6, FOXJ2, and HNRNPUH1 [2, 3, 4, 5, 6]. It has been demonstrated that MEF2D rearrangement conferred an inferior prognosis for ALL [7], but the clinical and genetic features of MEF2D-rearranged B-cell ALL still remained not well-defined. Herein, we reported two MEF2D-rearranged B-cell ALL patients presenting as the BL-like disease.

One 35-year-old female...



We thanked all members of the Zhejiang University Institute of Hematology and bone marrow morphology laboratory of the First Affiliated Hospital to Zhejiang University College of Medicine for technical support to diagnosis.

Author contributions

J.-W. S, W.-J. Y., and X.Z. designed the experiments. W.-J. Y. and X.Z. collected clinical specimens and integrated clinical materials. J.-W. S. displayed PCR experiment for verification. J.-W. S, W.-J. Y., and X.Z. integrated and analyzed data. J.-W. S and X. Z. wrote the manuscript. W.-J. Y. revised the manuscript. All authors approved the manuscript.

Funding information

This study was funded by the National Natural Science Foundation of China (81670124, 81800199).

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Statement of informed consent

Informed consent was obtained from all individual participants included in the study.

Supplementary material

277_2019_3857_MOESM1_ESM.docx (27 kb)
Table S1 (DOCX 27 kb)


  1. 1.
    Blum KA, Lozanski G, Byrd JC (2004) Adult Burkitt leukemia and lymphoma. Blood 104(10):3009–3020. CrossRefPubMedGoogle Scholar
  2. 2.
    Lilljebjorn H, Fioretos T (2017) New oncogenic subtypes in pediatric B-cell precursor acute lymphoblastic leukemia. Blood 130(12):1395–1401. CrossRefPubMedGoogle Scholar
  3. 3.
    Ohki K, Kiyokawa N, Saito Y, Hirabayashi S, Nakabayashi K, Ichikawa H, Momozawa Y, Okamura K, Yoshimi A, Ogata-Kawata H, Sakamoto H, Kato M, Fukushima K, Hasegawa D, Fukushima H, Imai M, Kajiwara R, Koike T, Komori I, Matsui A, Mori M, Moriwaki K, Noguchi Y, Park MJ, Ueda T, Yamamoto S, Matsuda K, Yoshida T, Matsumoto K, Hata K, Kubo M, Matsubara Y, Takahashi H, Fukushima T, Hayashi Y, Koh K, Manabe A, Ohara A, Tokyo Children's Cancer Study G (2019) Clinical and molecular characteristics of MEF2D fusion-positive B-cell precursor acute lymphoblastic leukemia in childhood, including a novel translocation resulting in MEF2D-HNRNPH1 gene fusion. Haematologica 104(1):128–137. CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Hirzel AC, Cotrell A, Gasparini R, Sriganeshan V (2013) Precursor B-cell acute lymphoblastic leukemia/lymphoma with L3 morphology, Philadelphia chromosome, MYC gene translocation, and coexpression of TdT and surface light chains: a case report. Case Rep Pathol 2013:679892. CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Li JF, Dai YT, Lilljebjorn H, Shen SH, Cui BW, Bai L, Liu YF, Qian MX, Kubota Y, Kiyoi H, Matsumura I, Miyazaki Y, Olsson L, Tan AM, Ariffin H, Chen J, Takita J, Yasuda T, Mano H, Johansson B, Yang JJ, Yeoh AE, Hayakawa F, Chen Z, Pui CH, Fioretos T, Chen SJ, Huang JY (2018) Transcriptional landscape of B cell precursor acute lymphoblastic leukemia based on an international study of 1,223 cases. Proc Natl Acad Sci U S A 115(50):E11711–E11720. CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Gu Z, Churchman M, Roberts K, Li Y, Liu Y, Harvey RC, McCastlain K, Reshmi SC, Payne-Turner D, Iacobucci I, Shao Y, Chen IM, Valentine M, Pei D, Mungall KL, Mungall AJ, Ma Y, Moore R, Marra M, Stonerock E, Gastier-Foster JM, Devidas M, Dai Y, Wood B, Borowitz M, Larsen EE, Maloney K, Mattano LA Jr, Angiolillo A, Salzer WL, Burke MJ, Gianni F, Spinelli O, Radich JP, Minden MD, Moorman AV, Patel B, Fielding AK, Rowe JM, Luger SM, Bhatia R, Aldoss I, Forman SJ, Kohlschmidt J, Mrozek K, Marcucci G, Bloomfield CD, Stock W, Kornblau S, Kantarjian HM, Konopleva M, Paietta E, Willman CL, Loh ML, Hunger SP, Mullighan CG (2016) Genomic analyses identify recurrent MEF2D fusions in acute lymphoblastic leukaemia. Nat Commun 7:13331. CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Suzuki K, Okuno Y, Kawashima N, Muramatsu H, Okuno T, Wang X, Kataoka S, Sekiya Y, Hamada M, Murakami N, Kojima D, Narita K, Narita A, Sakaguchi H, Sakaguchi K, Yoshida N, Nishio N, Hama A, Takahashi Y, Kudo K, Kato K, Kojima S (2016) MEF2D-BCL9 Fusion gene is associated with high-risk acute B-cell precursor lymphoblastic leukemia in adolescents. J Clin Oncol 34(28):3451–3459. CrossRefPubMedGoogle Scholar
  8. 8.
    Lou Y, Ma Y, Li C, Suo S, Tong H, Qian W, Mai W, Meng H, Yu W, Mao L, Wei J, Xu W, Jin J (2017) Efficacy and prognostic factors of imatinib plus CALLG2008 protocol in adult patients with newly diagnosed Philadelphia chromosome-positive acute lymphoblastic leukemia. Front Med 11(2):229–238. CrossRefPubMedGoogle Scholar
  9. 9.
    Lessard M, Fenneteau O, Sainty D, Valensi F, MacIntyre E, Flandrin G (1993) Translocation t(1;19) in acute lymphoblastic leukemia patients with cytological presentation simulating L3-ALL (Burkitt-like). Leuk Lymphoma 11(1-2):149–152. CrossRefPubMedGoogle Scholar
  10. 10.
    Grande BM, Gerhard DS, Jiang A, Griner NB, Abramson JS, Alexander TB, Allen H, Ayers LW, Bethony JM, Bhatia K, Bowen J, Casper C, Choi JK, Culibrk L, Davidsen TM, Dyer MA, Gastier-Foster JM, Gesuwan P, Greiner TC, Gross TG, Hanf B, Harris NL, He Y, Irvin JD, Jaffe ES, Jones SJM, Kerchan P, Knoetze N, Leal FE, Lichtenberg TM, Ma Y, Martin JP, Martin MR, Mbulaiteye SM, Mullighan CG, Mungall AJ, Namirembe C, Novik K, Noy A, Ogwang MD, Omoding A, Orem J, Reynolds SJ, Rushton CK, Sandlund JT, Schmitz R, Taylor C, Wilson WH, Wright GW, Zhao EY, Marra MA, Morin RD, Staudt LM (2019) Genome-wide discovery of somatic coding and noncoding mutations in pediatric endemic and sporadic Burkitt lymphoma. Blood 133(12):1313–1324. CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Center Laboratory, Affiliated Secondary HospitalZhejiang Chinese Medical UniversityHangzhouChina
  2. 2.Department of Hematology, The First Affiliated HospitalZhejiang University College of MedicineHangzhouChina
  3. 3.Institute of HematologyZhejiang UniversityHangzhouChina
  4. 4.Key Laboratory of Hematologic Malignancies, Diagnosis and Treatment, ZhejiangZhejiang University College of MedicineHangzhouChina

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