Primary Effusion Lymphoma in an Elderly HIV-Negative Patient with Hemodialysis: Importance of Evaluation for Pleural Effusion in Patients Receiving Hemodialysis

Abstract

Pleural effusion is a ubiquitous complication in hemodialysis (HD) patients. Common etiologies of pleural effusion in this patient group are heart failure, volume overload, parapneumonic effusion, tuberculotic pleuritis, and uremic pleuritis. Although thoracentesis is a useful diagnostic method of pleural effusion, empirical reduction of the dry weight is often attempted without thoracentesis because pleural effusion is commonly caused by volume overload and responds to the dry-weight reduction. However, this empiricism has a risk of overlooking or delaying the diagnosis of potentially fatal etiologies that need specific treatments. We report an 86-year-old human immunodeficiency virus (HIV)-negative male on HD with primary effusion lymphoma (PEL), a large-cell non-Hodgkin lymphoma presenting with characteristic lymphomatous effusions in the absence of solid tumor masses, which is in association with human herpes virus 8 (HHV8) infection in immunocompromised individuals. The patient presented with left-sided pleural effusion. This is the first case report of PEL developing in a patient receiving HD. Thoracentesis and cytological analysis of the effusion was key to the diagnosis. We also review the literature regarding pleural effusion in HD patients. Further, we examine Kaposi's sarcoma herpes virus/HHV8-negative effusion-based lymphoma, a newly proposed distinct lymphoma that clinically and cytomorphologically resembles PEL, because it can be cured without chemotherapy. This report may arouse clinicians' attention regarding the importance of evaluation for pleural effusion in HD patients, especially when the effusion or symptoms associated with pleural effusion are refractory to volume control.

Diagnostic significance of adenosine deaminase in pleural effusate for primary effusion lymphoma–like lymphoma

“…However, only 7 of the 47 cases that we reviewed described pleural fluid ADA levels. We have presented data on the ADA levels of a total of 9 cases in Table 1, including these 7 cases and our present and previous case 2‐7 …”

Primary effusion lymphoma: current perspectives

“… 5 PEL typically presents in middle-aged patients infected with HIV or harboring other immunocompromised states, such as recipients of solid-organ transplants, patients with cirrhosis, and in the elderly, often in HHV8 endemic areas. 6 – 10 Epstein Bar virus (EBV) co-infection is commonly found (60%–90% of cases) although its role in the pathogenesis of PEL is not clear. 11 EBV-negative PELs are typically found in elderly HIV-negative patients from HHV8-endemic areas.…”

T Cell Primary Effusion Lymphoma in an HIV-Negative Man with Liver Cirrhosis

“…Association with hepatitis C virus (HCV) has been documented [3], but there is no known association of HHV-8-positive PEL with alcohol-induced liver cirrhosis and PEL. Additionally, a similar association with end-stage renal disease and PEL has been described, indicating an association with volume overload states [9]. Only a few cases of PEL with T cell antigen expression have been recorded among HHV-8-positive patients [6].…”