PRIMARY EFFUSION LYMPHOMA: A CASE REPORT OF DIAGNOSIS IN PLEURAL FLUID
DOI:
https://doi.org/10.56238/levv16n53-029Keywords:
Primary Effusion Lymphoma, Pleural Effusion, HIV, HHV-8Abstract
Primary Effusion Lymphoma (PEL) is a rare and highly aggressive post-germinal center B-cell neoplasm characterized by predominantly developing in serous cavities, such as the pleura, without the initial formation of solid tumor masses. Diagnosis of this entity is challenging and requires confirmation of an etiological association with human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma herpesvirus. PEL is strongly linked to immunodeficiency states, with Human Immunodeficiency Virus (HIV) infection being the main epidemiological factor, conferring a poor prognosis and a median survival of less than two years. This article reports the case of a 29-year-old male patient, HIV-positive and previously diagnosed with Kaposi's sarcoma, who developed bilateral pleural effusion. Diagnostic investigation by thoracentesis revealed serohematic pleural fluid with high cellularity of atypical cells with an immunoblastic and plasmablastic pattern. Pathological, cytological, and immunohistochemical studies were crucial for the diagnosis, demonstrating positivity for HHV-8 (LANA-1), CD30, and CD45, as well as a high cell proliferation index with Ki-67 of approximately 95% and negative for pan-B and EBV markers. The clinical and pathological findings confirmed the diagnosis of primary effusion lymphoma in an immunocompromised patient. This case reinforces the epidemiological profile of PEL, which is more prevalent in HIV-positive men, and highlights the importance of HHV-8 coinfection, which plays a role in immune evasion and oncogenesis. Given the morphological overlap with other lymphoproliferative disorders, the use of a specific immunohistochemical panel, with emphasis on HHV-8 testing, is essential for accurate diagnosis. Case reports like this are vital for deepening the clinical and pathological understanding of this rare neoplasm, guiding investigation and therapeutic decisions, such as the EPOCH regimen initiated in this case, in patients with cavitary effusions of unclear etiology and immunocompromise.
Downloads
References
1. di Napoli A, Soma L, Quintanilla-Martinez L, de Leval L, Leoncini L, Zamò A, et al. Cavity-based lymphomas: challenges and novel concepts. A report of the 2022 EA4HP/SH lymphoma workshop. Virchows Archiv. 2023 Sep 1;483(3):299–316.
2. Hammock L, Reisenauer A, Wang W, Cohen C, Birdsong G, Folpe AL. Latency-associated nuclear antigen expression and human herpesvirus-8 polymerase chain reaction in the evaluation of Kaposi sarcoma and other vascular tumors in HIV-positive patients. Modern Pathology. 2005 Apr;18(4):463–8.
3. Cozzi I, Rossi G, Rullo E, Ascoli V. Classic KSHV/HHV-8-positive Primary Effusion Lymphoma (PEL): A Systematic Review and Meta-Analysis of Case Reports. Vol. 14, Mediterranean Journal of Hematology and Infectious Diseases. Universita Cattolica del Sacro Cuore; 2022.
4. Movva H, Rastgar Y, Obeidat K, Lewis A. A Rare Diagnosis of Primary Effusion Lymphoma Presenting With Three-Cavity Effusion: A Case Report. Cureus [Internet]. 2025 Aug 5; Available from: https://www.cureus.com/articles/377756-a-rare-diagnosis-of-primary-effusion-lymphoma-presenting-with-three-cavity-effusion-a-case-report
5. Antonangelo L, Vargas FS, Teixeira LR, Vaz MAC, Sales MM, Moreira LC, et al. Linfoma primário de cavidade pleural em paciente imunocompetente* Primary effusion lymphoma in an immunocompetent patient. Vol. 31, J Bras Pneumol. 2005.
6. Liu CY, Chen BJ, Chuang SS. Primary Effusion Lymphoma: A Timely Review on the Association with HIV, HHV8, and EBV. Vol. 12, Diagnostics. Multidisciplinary Digital Publishing Institute (MDPI); 2022.
7. Hu Z, Pan Z, Chen W, Shi Y, Wang W, Yuan J, et al. Primary effusion lymphoma: A clinicopathological study of 70 cases. Cancers. 2021 Feb 2;13(4):1–14.
8. Gathers DA, Galloway E, Kelemen K, Rosenthal A, Gibson SE, Munoz J. Primary Effusion Lymphoma: A Clinicopathologic Perspective. Vol. 14, Cancers. MDPI; 2022.
9. Statistics at a glance, 2022 Top 5 most frequent cancers Number of new cases 627 193 Number of deaths 278 835 Number of prevalent cases (5-year).
10. Ministério da Saúde Instituto Nacional de Câncer José Alencar Gomes da Silva Ministério da Saúde Instituto Nacional de Câncer. 2023.
11. Yu CJ, Damania B. Molecular Mechanisms of Kaposi Sarcoma-Associated Herpesvirus (HHV8)-Related Lymphomagenesis. Vol. 16, Cancers. Multidisciplinary Digital Publishing Institute (MDPI); 2024.
12. World Health Organization. HIV Data and Statistics [Internet]. www.who.int. 2024. Available from: https://www.who.int/teams/global-hiv-hepatitis-and-stis-programmes/hiv/strategic-information/hiv-data-and-statistics
13. Lima L, Costa M, Silva A, Evangelista T, Lopes A, Vieira J, et al. LINFOMA EXTRANODAL NK/T CELL NASAL: RELATO DE CASO CLÍNICO. Hematology, Transfusion and Cell Therapy. 2024 Oct;46:S265.