Disseminated Double-Hit Lymphoma in a Young Adult
DOI:
https://doi.org/10.21141/PJP.2021.07Keywords:
lymphoma, B-cell lymphoma, molecular diagnostic techniqueAbstract
High-grade B-cell lymphoma with MYC and BCL2 and-or BCL6 rearrangements, also called double-hit lymphoma, is an aggressive mature B-cell lymphoma that carries both MYC and BCL2 and/or BCL6 translocations. This can present as a diagnostic challenge since clinical characteristics, morphology and immunophenotype are not accurate indicators of underlying genetic aberrations in this type of lymphoma. We report a case of a 25-year-old male who presented with a one-year history of cough, gradually increasing abdominal girth and jaundice. Definitive diagnosis was made post-mortem with additional ancillary studies using immunohistochemistry staining and fluorescent in-situ hybridization studies.
Downloads
Download data is not yet available.
References
1. Swerdlow SH, Campo E, Harris NL, et al, eds. WHO classification of tumours of haematopoietic and lymphoid tissues, rev. 4th ed, vol. 2. Lyon, International Agency for Research on Cancer; 2017.
2. Smith SM. Aggressive B-cell lymphoma: the double-hit and double-expresser phenotypes. Clin Adv Hematol Oncol. 2017;15(1):40-2. https://pubmed.ncbi.nlm.nih.gov/28212368.
3. Stephens DM, Smith SM. Diffuse large B-cell lymphoma- who should we FISH? Ann Lymphoma. 2018; 2:1-10. https://doi.org/10.21037/aol.2018.11.01.
4. Aukema SM, Siebert R, Schuuring E, et al. Double-hit-B-cell lymphoma. Blood. 2011; 117(8):2319-31. https://pubmed.ncbi.nlm.nih.gov/21119107. https://doi.org/10.1182/blood-2010-09-297879.
5. Msimang MZ, Ramdial PK, Kuppusamy JB, Nargan K, Sheik-Gafoor MH. AIDS-associated pediatric high-grade B-cell lymphoma with MYC and BCL2 translocations. J AIDS Clin Res. 2018;8(11):742. https://doi.org/10.4172/2155-6113.1000742.
6. Niitsu N, Okamoto M, Miura I. et al. Clinical features and prognosis of de novo diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC translocations. Leukemia. 2009;23(4):777–83. https://pubmed.ncbi.nlm.nih.gov/19151788. https://doi.org/10.1038/leu.2008.344.
7. López-Guillermo A, Colomo L, Jímenez M, et al. Diffuse large B-cell lymphoma: clinical and biological characterization and outcome according to the nodal or extranodal primary origin. J Clin Oncol. 2016; 23(12):2797-804. https://pubmed.ncbi.nlm.nih.gov/15728226. https://doi.org/10.1200/JCO.2005.07.155.
8. Oliveira CC, Maciel-Guerra H, Kucko L, et al. Double-hit lymphomas: clinical, morphological, immunohistochemical and cytogenetic study in a series of Brazilian patients with high-grade non-Hodgkin lymphoma. Diagn Pathol. 2017;12(1):3. https://pubmed.ncbi.nlm.nih.gov/28061782.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5219810. https://doi.org/10.1186/s13000-016-0593-0.
9. Friedberg, JW. How I treat double hit lymphoma. Blood. 2017;130(5): 590-6. https://pubmed.ncbi.nlm.nih.gov/28600333. https://doi.org/10.1182/blood-2017-04-737320.
10. Liu Y, Barta SK. Diffuse large B-cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment. Am J Hematol. 2019;95(5):604-16. https://pubmed.ncbi.nlm.nih.gov/30859597. https://doi.org/10.1002/ajh.25460.
11. Kim H, Kim HJ, Kim SH. Diagnostic approach for double-hit and triple-hit lymphoma based on immunophenotypic and cytogenetic characteristics of bone marrow specimens. Ann Lab Med. 2020;40(5):361-9. https://pubmed.ncbi.nlm.nih.gov/32311849.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7169621. https://doi.org/10.3343/alm.2020.40.5.361.
12. Friedberg JW. Double hit diffuse large B-cell lymphomas: diagnostic and therapeutic challenges. Chin Clin Oncol. 2015; 4(1):9. https://pubmed.ncbi.nlm.nih.gov/25841716. https://doi.org/10.3978/j.issn.2304-3865.2015.03.02.
13. Davies, A. Double-hit lymphoma: so what? Hematol Oncol. 2019; 37(Suppl 1):19-23. https://pubmed.ncbi.nlm.nih.gov/31187528. https://doi.org/10.1002/hon.2581.
14. Di Napoli A, Remotti D, Agostinelli C, et al. A practical algorithmic approach to mature aggressive B cell lymphoma diagnosis in the double/triple hit era: selecting cases, matching clinical benefit. Virchows Archiv. 2019;475(4):513–8. https://pubmed.ncbi.nlm.nih.gov/31388760.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6828632. https://doi.org/10.1007/s00428-019-02637-2.
15. Ambrosio MR, Lazzi S, Lo Bello G, et al. MYC protein expression scoring and its impact on the prognosis of aggressive B-cell lymphoma patients. Haematologica. 2019;104(1):e25-8. https://pubmed.ncbi.nlm.nih.gov/29954940.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312021. https://doi.org/10.3324/haematol.2018.195958.
16. Phuoc V, Sus J, Chavez JC. Drug therapy for double-hit lymphoma. Drugs Context. 2019;8:2019-8-1. https://pubmed.ncbi.nlm.nih.gov/31844420.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6905641. https://doi.org/10.7573/dic.2019-8-1.
2. Smith SM. Aggressive B-cell lymphoma: the double-hit and double-expresser phenotypes. Clin Adv Hematol Oncol. 2017;15(1):40-2. https://pubmed.ncbi.nlm.nih.gov/28212368.
3. Stephens DM, Smith SM. Diffuse large B-cell lymphoma- who should we FISH? Ann Lymphoma. 2018; 2:1-10. https://doi.org/10.21037/aol.2018.11.01.
4. Aukema SM, Siebert R, Schuuring E, et al. Double-hit-B-cell lymphoma. Blood. 2011; 117(8):2319-31. https://pubmed.ncbi.nlm.nih.gov/21119107. https://doi.org/10.1182/blood-2010-09-297879.
5. Msimang MZ, Ramdial PK, Kuppusamy JB, Nargan K, Sheik-Gafoor MH. AIDS-associated pediatric high-grade B-cell lymphoma with MYC and BCL2 translocations. J AIDS Clin Res. 2018;8(11):742. https://doi.org/10.4172/2155-6113.1000742.
6. Niitsu N, Okamoto M, Miura I. et al. Clinical features and prognosis of de novo diffuse large B-cell lymphoma with t(14;18) and 8q24/c-MYC translocations. Leukemia. 2009;23(4):777–83. https://pubmed.ncbi.nlm.nih.gov/19151788. https://doi.org/10.1038/leu.2008.344.
7. López-Guillermo A, Colomo L, Jímenez M, et al. Diffuse large B-cell lymphoma: clinical and biological characterization and outcome according to the nodal or extranodal primary origin. J Clin Oncol. 2016; 23(12):2797-804. https://pubmed.ncbi.nlm.nih.gov/15728226. https://doi.org/10.1200/JCO.2005.07.155.
8. Oliveira CC, Maciel-Guerra H, Kucko L, et al. Double-hit lymphomas: clinical, morphological, immunohistochemical and cytogenetic study in a series of Brazilian patients with high-grade non-Hodgkin lymphoma. Diagn Pathol. 2017;12(1):3. https://pubmed.ncbi.nlm.nih.gov/28061782.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5219810. https://doi.org/10.1186/s13000-016-0593-0.
9. Friedberg, JW. How I treat double hit lymphoma. Blood. 2017;130(5): 590-6. https://pubmed.ncbi.nlm.nih.gov/28600333. https://doi.org/10.1182/blood-2017-04-737320.
10. Liu Y, Barta SK. Diffuse large B-cell lymphoma: 2019 update on diagnosis, risk stratification, and treatment. Am J Hematol. 2019;95(5):604-16. https://pubmed.ncbi.nlm.nih.gov/30859597. https://doi.org/10.1002/ajh.25460.
11. Kim H, Kim HJ, Kim SH. Diagnostic approach for double-hit and triple-hit lymphoma based on immunophenotypic and cytogenetic characteristics of bone marrow specimens. Ann Lab Med. 2020;40(5):361-9. https://pubmed.ncbi.nlm.nih.gov/32311849.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7169621. https://doi.org/10.3343/alm.2020.40.5.361.
12. Friedberg JW. Double hit diffuse large B-cell lymphomas: diagnostic and therapeutic challenges. Chin Clin Oncol. 2015; 4(1):9. https://pubmed.ncbi.nlm.nih.gov/25841716. https://doi.org/10.3978/j.issn.2304-3865.2015.03.02.
13. Davies, A. Double-hit lymphoma: so what? Hematol Oncol. 2019; 37(Suppl 1):19-23. https://pubmed.ncbi.nlm.nih.gov/31187528. https://doi.org/10.1002/hon.2581.
14. Di Napoli A, Remotti D, Agostinelli C, et al. A practical algorithmic approach to mature aggressive B cell lymphoma diagnosis in the double/triple hit era: selecting cases, matching clinical benefit. Virchows Archiv. 2019;475(4):513–8. https://pubmed.ncbi.nlm.nih.gov/31388760.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6828632. https://doi.org/10.1007/s00428-019-02637-2.
15. Ambrosio MR, Lazzi S, Lo Bello G, et al. MYC protein expression scoring and its impact on the prognosis of aggressive B-cell lymphoma patients. Haematologica. 2019;104(1):e25-8. https://pubmed.ncbi.nlm.nih.gov/29954940.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312021. https://doi.org/10.3324/haematol.2018.195958.
16. Phuoc V, Sus J, Chavez JC. Drug therapy for double-hit lymphoma. Drugs Context. 2019;8:2019-8-1. https://pubmed.ncbi.nlm.nih.gov/31844420.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6905641. https://doi.org/10.7573/dic.2019-8-1.
Published
2021-06-29
How to Cite
Damian, K., & Cancio, J. E. . (2021). Disseminated Double-Hit Lymphoma in a Young Adult. PJP, 6(1), 56–61. https://doi.org/10.21141/PJP.2021.07
Issue
Section
Autopsy Vault
License
The Philippine Journal of Pathology is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License. Based on works made open access at http://philippinejournalofpathology.org
@philippinepathologyjournal