A CASE REPORT WITH FIBRIN-ASSOCIATED DIFFUSE LARGE B-CELL LYMPHOMA SECONDARY TO CARDIAC MYXOMA

Year 2021, Volume: 8 Issue: 2, 91 - 94, 30.06.2021

Fatih Erkan Akay , Nurija Bilaović

Abstract

Aims: To raise awareness for differential diagnosis of fibrin-associated diffuse large B-cell lymphoma with patients that have sustained chronic inflammation or are immunocompetent with a previous Epstein-Barr virus infection. Case Report: A 58-year-old male patient was admitted to the Clinical Center of Sarajevo University, Cardiovascular Surgery Department with the symptoms of getting tired quickly accompanied by dyspnea. His echocardiography findings exhibited a large polymorphic clavicle type highly mobile formation in his left atrium with a size of 76x23mm, intermittently prolapsing the annulus of the mitral valve and reaching the middle of the extended left ventricle. After the detection of a cardiac mass, the patient underwent surgery and had a total excision of the mass. His- topathological analysis showed a tumor made of stellate cells that form complex structures resembling wires together with an islet of plasma cells embedded in a myxoid/fibrinoid background. Immunohistochemically, lymphoma cells were positively stained for CD20, CD30, MUM1, and EBER. After excluding all other systemic manifestations of any other diseases, the patient was diagnosed with fibrin-associated diffuse large B-cell lymphoma, as a primary cardiac lymphoma, and myxoma. Conclusion: In conclusion, we are reporting a very rare case seen approximately 3% of all lymphomas in the Western Population associated with Epstein-Barr virus B-cell Lymphoproliferative disorders, therefore making them harder to diagnose due to limited experience. Albeit being an infrequent disease fibrin-associated diffuse large B-cell lymphoma should be an entity included in the differential diagnosis of the patients that have sustained chronic inflammation or are immunocompetent with a previous Epstein-Barr virus infection.

Keywords

Cardiac myxoma, diffuse large B-cell lymphoma, Epstein-Barr virus infection

References

• 1. Gowda RM, Khan IA. Clinical perspectives of primary cardiac lymphoma. Angi- ology 2003;54(5):599-604.
• 2. Swerdlow SH, Campo E, Harris NL et al. WHO classification of tumours of hae- matopoietic and lymphoid tissues. 2nd ed. In: Swerdlow SH, Campo E, Harris NL et al, editors. Geneva: WHO Press; 2008.p.311.
• 3. Nascimento AF, Winters GL, Pinkus GS. Primary cardiac lymphoma: clinical, his- tologic, immunophenotypic, and genotypic features of 5 cases of a rare disorder. Am J Surg Pathol 2007;31(9):1344-50.
• 4. Basso C, Rizzo S, Valente M et al. Cardiac masses and tumours. Heart 2016;102(15):1230-45.
• 5. O'Mahony D, Peikarz RL, Bandettini WP et al. Cardiac involvement with lym- phoma: a review of the literature. Clin Lymphoma Myeloma 2008;8(4):249-52.
• 6. Antoniades L, Eftychiou C, Petrou PM et al. Primary cardiac lymphoma: case report and brief review of the literature. Echocardiography 2009;26(2):214-9.
• 7. Dojcinov SD, Fend F, Quintanilla-Martinez L. EBV-Positive lymphoproliferations of B- T- and NK-cell derivation in non-immunocompromised hosts. Pathogens 2018;7(1):713.
• 8. Giunta R, Cravero RG, Granata G et al. Primary cardiac T-cell lymphoma. Ann Hematol 2004;83(7):450-4.
• 9. Boyer DF, McKelvie PA, de Leval L et al. Fibrin-associated EBV-positive large B-Cell lymphoma: an indolent neoplasm with features distinct from diffuse large B-Cell lymphoma associated with chronic inflammation. Am J Surg Pathol 2017;41(3):299-312.
• 10. Kim H-J, Ko YH, Kim JE et al. Epstein-Barr virus-associated lymphoproliferative disorders: review and update on 2016 WHO classification. J Pathol Transl Med 2017;51(4):352-8.
• 11. Dolcetti R, Giunco S, Dal Col J et al. Epstein-Barr virus and telomerase: from cell immortalization to therapy. Infect Agent Cancer 2014;9(1):8.
• 12. Kamranvar SA, Chen X, Masucci MG. Telomere dysfunction and activation of alternative lengthening of telomeres in B-lymphocytes infected by Epstein-Barr virus. Oncogene 2013;32(49):5522-30.
• 13. Chen BJ, Chapuy B, Ouyang J et al. PD-L1 expression is characteristic of a subset of aggressive B-cell lymphomas and virus-associated malignancies. Clin Cancer Res 2013;19(13):3462-73.
• 14. Loong F, Chan AC, Ho BC et al. Diffuse large B-cell lymphoma associated with chronic inflammation as an incidental finding and new clinical scenarios. Mod Pathol 2010;23(4):493-501.
• 15. Hubackova S, Krejcikova K, Bartek J et al. Interleukin 6 signaling regulates pro- myelocytic leukemia protein gene expression in human normal and cancer cells. J Biol Chem 2012;287(32):26702-14.
• 16. Faganello G, Belham M, Thaman R et al. A case of primary cardiac lymphoma: analysis of the role of echocardiography in early diagnosis. Echocardiography 2007;24(8):889-92.
• 17. Nonami A, Takenaka K, Kamezaki K et al. Successful treatment of primary cardi- ac lymphoma by rituximab-CHOP and high-dose chemotherapy with autologous peripheral blood stem cell transplantation. Int J Hematol 2007;85(3):264-6.