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CC BY 4.0 · Indian J Med Paediatr Oncol 2024; 45(04): 346-350
DOI: 10.1055/s-0043-1772233
Case Report with Review of Literature

Double-Expressor Lymphoma in a Young Child—A Case Report and Review of Literature

Anjali Shaju
1   Department of Pediatrics, Sri Ramachandra Institute of Higher Education and Research, Porur, Chennai, Tamil Nadu, India
,
Latha M. Sneha
2   Department of Pediatric Hematology and Oncology, Sri Ramachandra Institute of Higher Education and Research, Porur, Chennai, Tamil Nadu, India
,
Nidarshana Pandian
1   Department of Pediatrics, Sri Ramachandra Institute of Higher Education and Research, Porur, Chennai, Tamil Nadu, India
,
Suresh Chandra
3   Histopathology, Medgenome, Bangalore, Karnataka, India
,
Sonam Poonam Nisar
4   Department of Orbit, Oculoplasty, Reconstructive and Aesthetic Services, Sankara Nethralaya, Chennai, Tamil Nadu, India
,
Krishnakumar Subramanian
5   Pathology Department, Vision Research Foundation, Chennai, Tamil Nadu, India
,
Priyathersini Nagarajan 
6   Department of Pathology, Sri Ramachandra Institute of Higher Education and Research, Porur, Chennai, Tamil Nadu, India
› Author Affiliations Funding The authors received no financial support for the research, authorship, and/or publication of this article.
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Abstract

Double hit lymphoma (DHL) and double-expressor lymphoma (DEL) are now considered as aggressive types of diffuse large B cell lymphoma. DHL is characterized by a dual rearrangement of MYC and B cell lymphoma 2 (BCL-2) and/or B cell lymphoma 6 (BCL-6) and DEL by overexpression of MYC and BCL-2. Both DHL and DEL have aggressive presentation and are more common in elderly population. We present a case of 1 ½ years old boy who presented with bilateral proptosis, and diagnosed as non-Hodgkin lymphoma with central nervous system involvement. Immunohistochemistry revealed high expression of MYC and BCL-2. Fluorescence in situ hybridization studies done to rule out DHL showed no translocation of C-MYC, Bcl-2, and Bcl-6 and hence were confirmed as double-expressor high-grade B cell lymphoma. Dual expression of C- MYC, Bcl-2, or Bcl-6 always needs further evaluation to rule out the more aggressive DHL subtypes.

Keywords

child - proptosis - non-Hodgkin lymphoma - double-expressor lymphoma

Authors’ Contributions

Latha MS conceptualized the report, performed literature review, and edited the manuscript. Anjali Shaju and Nidarshana Pandian drafted the manuscript. Krishnakumar, Suresh Chandra, Priyathersini, and Sonam Poonam edited and revised the manuscript. All authors read and approved the manuscript for publication.


Patient's Consent

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




Publication History

Article published online:
22 September 2023

© 2023. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

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  • References

  • 1 Riedell PA, Smith SM. Double hit and double expressors in lymphoma: definition and treatment. Cancer 2018; 124 (24) 4622-4632
    CrossrefPubMedGoogle Scholar
  • 2 Swerdlow SH, Campo E, Pileri SA. et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127 (20) 2375-2390
    CrossrefPubMedGoogle Scholar
  • 3 Friedberg JW. Double-hit diffuse large B-cell lymphoma. J Clin Oncol 2012; 30 (28) 3439-3443
    CrossrefPubMedGoogle Scholar
  • 4 Nguyen L, Papenhausen P, Shao H. The role of c-MYC in B-cell lymphomas: diagnostic and molecular aspects. Genes (Basel) 2017; 8 (04) 116
    CrossrefPubMedGoogle Scholar
  • 5 Nagib RM, Ibrahim EM, El-Ashwah S. Double-expressor and triple-expressor lymphomas: are these prognostically distinct groups of diffuse large B-cell lymphoma?. Egypt J Pathol 2019; 39: 239-243
    CrossrefGoogle Scholar
  • 6 Karube K, Campo E. MYC alterations in diffuse large B-cell lymphomas. Semin Hematol 2015; 52 (02) 97-106
    CrossrefPubMedGoogle Scholar
  • 7 Rosenwald A, Bens S, Advani R. et al. Prognostic significance of MYC Rearrangement and Translocation partner in diffuse large B-cell lymphoma—a study by the Lunenburg lymphoma biomarker consortium. J Clin Oncol 2019; 37 (35) 3359-3368
    CrossrefPubMedGoogle Scholar
  • 8 Riedell PA, Smith SM. Double hit and double expressors in lymphoma: definition and treatment. Cancer 2018; 124 (24) 4622-4632
    CrossrefPubMedGoogle Scholar
  • 9 Landsburg DJ, Petrich AM, Abramson JS. et al. Impact of oncogene rearrangement patterns on outcomes in patients with double-hit non-Hodgkin lymphoma. Cancer 2016; 122 (04) 559-564
    CrossrefPubMedGoogle Scholar
  • 10 Hu S, Xu-Monette ZY, Tzankov A. et al. MYC/BCL2 protein coexpression contributes to the inferior survival of activated B-cell subtype of diffuse large B-cell lymphoma and demonstrates high-risk gene expression signatures: a report from The International DLBCL Rituximab-CHOP Consortium Program. Blood 2013; 121 (20) 4021-4031 , quiz 4250
    CrossrefPubMedGoogle Scholar
  • 11 Oschlies I, Salaverria I, Mahn F. et al. Pediatric follicular lymphoma–a clinico-pathological study of a population-based series of patients treated within the non-Hodgkin's lymphoma–Berlin-Frankfurt-Munster (NHL-BFM) multicenter trials. Haematologica 2010; 95 (02) 253-259
    CrossrefPubMedGoogle Scholar
  • 12 Casulo C, Burack WR, Friedberg JW. Transformed follicular non-Hodgkin lymphoma. Blood 2015; 125 (01) 40-47
    CrossrefPubMedGoogle Scholar
  • 13 Davies A. Double-hit lymphoma: so what?. Hematol Oncol 2019; 37 (Suppl. 01) 19-23
    CrossrefPubMedGoogle Scholar
  • 14 Savage KJ, Slack GW, Mottok A. et al. Impact of dual expression of MYC and BCL2 by immunohistochemistry on the risk of CNS relapse in DLBCL. Blood 2016; 127 (18) 2182-2188
    CrossrefPubMedGoogle Scholar
  • 15 Green TM, Young KH, Visco C. et al. Immunohistochemical double-hit score is a strong predictor of outcome in patients with diffuse large B-cell lymphoma treated with rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone. J Clin Oncol 2012; 30 (28) 3460-3467
    CrossrefPubMedGoogle Scholar
  • 16 Hatzl S, Posch F, Deutsch A. et al. Immunohistochemistry for c-myc and bcl-2 overexpression improves risk stratification in primary central nervous system lymphoma. Hematol Oncol 2020; 38 (03) 277-283
    CrossrefPubMedGoogle Scholar
  • 17 Landsburg DJ, Falkiewicz MK, Maly J. et al. Outcomes of patients with double-hit lymphoma who achieve first complete remission. J Clin Oncol 2017; 35 (20) 2260-2267
    CrossrefPubMedGoogle Scholar
  • 18 Bartlett NL, Wilson WH, Jung SH. et al. Dose-adjusted EPOCH-R compared with R-CHOP as frontline therapy for diffuse large B-cell lymphoma: clinical outcomes of the phase III intergroup trial alliance/CALGB 50303. J Clin Oncol 2019; 37 (21) 1790-1799
    CrossrefPubMedGoogle Scholar
  • 19 Petrich AM, Gandhi M, Jovanovic B. et al. Impact of induction regimen and stem cell transplantation on outcomes in double-hit lymphoma: a multicenter retrospective analysis. Blood 2014; 124 (15) 2354-2361
    CrossrefPubMedGoogle Scholar
  • 20 Wu D, Wood BL, Dorer R, Fromm JR. “Double-Hit” mature B-cell lymphomas show a common immunophenotype by flow cytometry that includes decreased CD20 expression. Am J Clin Pathol 2010; 134 (02) 258-265
    CrossrefPubMedGoogle Scholar
  • 21 Hwang J, Suh C, Kim K. et al. The incidence and treatment response of double expression of MYC and BCL2 in patients with diffuse large B-cell lymphoma: a systematic review and meta-analysis. Cancers (Basel) 2021; 13 (13) 3369
    CrossrefPubMedGoogle Scholar
  • 22 Bharatnur SS, Amirtham U, Premalata CS, Kumar RV. Distribution of non-Hodgkin lymphoma in children and adolescents. A study from a regional cancer centre in South India. IOSR Journal of Dental and Medical Sciences 2016; 15 (04) 7-12 . e-ISSN: 2279–0853
    Google Scholar
  • 23 Biswas BK, Mondal R, Moulik D, Bagdi S. Primary ovarian non-Hodgkin's lymphoma in a 1-year-old child: a rare case report. Indian J Case Reports 2020; 6 (04) 166-168
    CrossrefGoogle Scholar