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Double-hit pancreatic B-lymphoblastic lymphoma with a variant translocation t(2;18)(p11;q21)

  • Katsuya YamamotoEmail author
  • Shinichiro Kawamoto
  • Akihito Kitao
  • Yu Mizutani
  • Yumiko Inui
  • Kimikazu Yakushijin
  • Kazuyoshi Kajimoto
  • Yoshitake Hayashi
  • Hiroshi Matsuoka
  • Hironobu Minami
Case Report

Abstract

Double-hit lymphoma is typically categorized as “high-grade B-cell lymphoma, with MYC and BCL2 and/or BCL6 rearrangements”, but in infrequent cases in which terminal deoxynucleotidyl transferase (TdT) expression is positive, it is categorized as B-lymphoblastic lymphoma (B-LBL). BCL2 rearrangements are usually caused by t(14;18)(q32;q21); variant translocations are very rare. Here, we describe an unusual case of double-hit pancreatic B-LBL with a variant translocation t(2;18)(p11;q21). A 69-year-old man was admitted because of an abdominal mass. Computed tomography scans demonstrated a diffusely enlarged pancreas and massive ascites. Cell block preparations of ascites cells revealed marked proliferation of blastic lymphoid cells positive for CD19, CD10, CD79a, PAX5, and TdT, indicating a diagnosis of B-LBL. G-banding and spectral karyotyping showed 45,XY,+X,t(2;18)(p11;q21),-4,der(5)t(1;5)(q12;p15),der(6)t(6;21)(q21;q?),t(8;14)(q24;q32),-15. Fluorescence in situ hybridization detected split BCL2 and IGH/MYC fusion signals. Almost all ascites cells were diffusely and strongly positive for MYC and BCL2. The patient died of progressive disease 20 days after admission. To our knowledge, this is the first reported case of MYC and BCL2 double-hit B-LBL with t(2;18)(p11;q21). High coexpression of MYC by t(8;14) and BCL2 by t(2;18) may be implicated in the development of B-LBL. Furthermore, double-hit B-LBL may be associated with a less favorable outcome compared with typical B-LBL.

Keywords

Double-hit lymphoma Variant translocation t(2;18)(p11;q21) B-lymphoblastic lymphoma Primary pancreatic lymphoma 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Merron B, Davies A. Double hit lymphoma: how do we define it and how do we treat it? Best Pract Res Clin Haematol. 2018;31:233–40.CrossRefGoogle Scholar
  2. 2.
    Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127:2375–90.CrossRefGoogle Scholar
  3. 3.
    Johnson NA, Savage KJ, Ludkovski O, Ben-Neriah S, Woods R, Steidl C, et al. Lymphomas with concurrent BCL2 and MYC translocations: the critical factors associated with survival. Blood. 2009;114:2273–9.CrossRefGoogle Scholar
  4. 4.
    McPhail ED, Maurer MJ, Macon WR, Feldman AL, Kurtin PJ, Ketterling RP, et al. Inferior survival in high-grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements is not associated with MYC/IG gene rearrangements. Haematologica. 2018;103:1899–907.CrossRefGoogle Scholar
  5. 5.
    Mitelman F, Johansson B, Mertens F, editors. Mitelman database of chromosome aberrations and gene fusions in cancer. (2019). https://cgap.nci.nih.gov/Chromosomes/Metelman. Accessed 20 Jan 2019.
  6. 6.
    Aventin A, Mecucci C, Guanyabens C, Brunet S, Soler J, Bordes R, et al. Variant t(2;18) translocation in a Burkitt conversion of follicular lymphoma. Br J Haematol. 1990;74:367–9.CrossRefGoogle Scholar
  7. 7.
    Hillion J, Mecucci C, Aventin A, Leroux D, Wlodarska I, Van Den Berghe H, et al. A variant translocation t(2;18) in follicular lymphoma involves the 5′ end of bcl-2 and Igκ light chain gene. Oncogene. 1991;6:169–72.Google Scholar
  8. 8.
    Juneja S, Matthews J, Lukeis R, Laidlaw C, Cooper I, Wolf M, et al. Prognostic value of cytogenetic abnormalities in previously untreated patients with non-Hodgkin’s lymphoma. Leuk Lymphoma. 1997;25:493–501.CrossRefGoogle Scholar
  9. 9.
    Henderson LJ, Okamoto I, Lestou VS, Ludkovski O, Robichaud M, Chhanabhai M, et al. Delineation of a minimal region of deletion at 6q16.3 in follicular lymphoma and construction of a bacterial artificial chromosome contig spanning a 6-megabase region of 6q16–q21. Genes Chromosmes Cancer. 2004;40:60–5.CrossRefGoogle Scholar
  10. 10.
    Tomita N, Tokunaka M, Nakamura N, Takeuchi K, Koike J, Motomura S, et al. Clinicopathological features of lymphoma/leukemia patients carrying both BCL2 and MYC translocations. Haematologica. 2009;94:935–43.CrossRefGoogle Scholar
  11. 11.
    Bassan R, Maino E, Cortelazzo S. Lymphoblastic lymphoma: an updated review on biology, diagnosis, and treatment. Eur J Haematol. 2016;96:447–60.CrossRefGoogle Scholar
  12. 12.
    Kaplan A, Samad A, Dolan MM, Cioc AM, Holman CJ, Schmechel SC, et al. Follicular lymphoma transformed to “double-hit” B lymphoblastic lymphoma presenting in the peritoneal fluid. Diagn Cytopathol. 2013;41:986–90.CrossRefGoogle Scholar
  13. 13.
    Geyer JT, Subramaniyam S, Jiang Y, Elemento O, Ferry JA, de Leval L, et al. Lymphoblastic transformation of follicular lymphoma: a clinicopathologic and molecular analysis of 7 patients. Hum Pathol. 2015;46:260–71.CrossRefGoogle Scholar
  14. 14.
    Liu W, Hu S, Konopleva M, Khoury JD, Kalhor N, Tang G, et al. De Novo MYC and BCL2 double-hit B-cell precursor acute lymphoblastic leukemia (BCP-ALL) in pediatric and young adult patients associated with poor prognosis. Pediatr Hematol Oncol. 2015;32:535–47.CrossRefGoogle Scholar
  15. 15.
    Kelemen K, Holden J, Johnson LJ, Davion S, Robetorye RS. Immunophenotypic and cytogenetic findings of B-lymphoblastic leukemia/lymphoma associated with combined IGH/BCL2 and MYC rearrangement. Cytom B Clin Cytom. 2017;92:310–4.CrossRefGoogle Scholar
  16. 16.
    Moench L, Sachs Z, Aasen G, Dolan M, Dayton V, Courville EL. Double- and triple-hit lymphomas can present with features suggestive of immaturity, including TdT expression, and create diagnostic challenges. Leuk Lymphoma. 2016;57:2626–35.CrossRefGoogle Scholar
  17. 17.
    Ok CY, Medeiros LJ, Thakral B, Tang G, Jain N, Jabbour E, et al. High-grade B-cell lymphomas with TdT expression: a diagnostic and classification dilemma. Mod Pathol. 2019;32:48–58.CrossRefGoogle Scholar
  18. 18.
    Saif MW. Primary pancreatic lymphomas. JOP. 2006;7:262–73.Google Scholar
  19. 19.
    Daniel SV, Vani DH, Smith AM, Hill QA, Menon KV. Obstructive jaundice due to a pancreatic mass: a rare presentation of acute lymphoblastic leukemia in an adult. JOP. 2010;11:72–4.Google Scholar
  20. 20.
    Nakaji S, Hirata N, Shiratori T, Kobayashi M, Fujii H, Ishii E, et al. A case of primary pancreatic lymphoblastic lymphoma diagnosed by endoscopic ultrasound-guided fine-needle aspiration. Clin J Gastroenterol. 2014;7:180–4.CrossRefGoogle Scholar

Copyright information

© Japanese Society of Hematology 2019

Authors and Affiliations

  • Katsuya Yamamoto
    • 1
    Email author
  • Shinichiro Kawamoto
    • 1
  • Akihito Kitao
    • 1
  • Yu Mizutani
    • 1
  • Yumiko Inui
    • 1
  • Kimikazu Yakushijin
    • 1
  • Kazuyoshi Kajimoto
    • 2
    • 3
  • Yoshitake Hayashi
    • 2
  • Hiroshi Matsuoka
    • 1
  • Hironobu Minami
    • 1
  1. 1.Division of Medical Oncology/Hematology, Department of MedicineKobe University Graduate School of MedicineKobeJapan
  2. 2.Division of Molecular Medicine and Medical Genetics, Department of PathologyKobe University Graduate School of MedicineKobeJapan
  3. 3.Department of PathologyHyogo Cancer CenterAkashiJapan

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