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

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          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.

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          Most cited references12

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          KSHV/HHV8-negative effusion-based lymphoma, a distinct entity associated with fluid overload states.

          Human herpesvirus-8 (HHV8)-positive effusion-based lymphomas have been termed primary effusion lymphoma (PEL) in the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Kaposi sarcoma herpesvirus (KSHV)/HHV8-negative effusion-based lymphomas (KSHV/HHV8-negative EBLs) resembling PELs have been reported in the literature and in many cases have been (mis)classified as PEL-like lymphomas. Herein, we present a series of cases and a review of KSHV/HHV8-negative EBLs. This lymphoma, although cytomorphologically resembling PEL, is a distinct entity with characteristic clinical and pathologic features. Patients are older, generally human immunodeficiency virus negative and not immunosuppressed, frequently hepatitis C positive compared with the population baseline, and often have an underlying medical condition leading to fluid overload. The lymphoma cells express pan-B-cell antigens in 86.7%, and CD20 is expressed in 71.1% of the cases. The lymphoma is often of germinal center B or mixed germinal center B/activated B-cell signature with the Hans classifier, and Epstein-Barr virus is positive in nearly 30% of cases. Rare T-cell lymphomas were also reported. Clinical outcomes and response to therapy, including isolated aspiration, are relatively favorable compared with cases of PEL. We suggest that HHV8-negative effusion-based lymphoma is a distinct entity associated with fluid overload states.
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            Disappearance of malignant cells by effusion drainage alone in two patients with HHV-8-unrelated HIV-negative primary effusion lymphoma-like lymphoma.

            Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin lymphoma usually confined to the body cavities of predominantly immunosuppressed patients infected with human herpes virus-8 (HHV-8). In PEL, malignant cells are usually negative for B-cell markers, such as CD19, CD20, and CD79a, but are positive for activation and plasma cell-related markers, such as CD30, CD38, and CD138. It has been reported that HHV-8-unrelated PEL shows high expression of B-cell markers, which is referred to as PEL-like lymphoma. Here, we report two cases of HHV-8-unrelated HIV-negative PEL-like lymphoma in which malignant cells regressed spontaneously after effusion drainage alone. These cells expressed B-cell markers, such as CD20. No chemotherapy was given to either patient, as they were of an advanced age, and both achieved a complete response by effusion drainage alone. One showed a complete response for 16 months after effusion drainage, and the other has survived for 11 months with a complete response. This suggests that sufficient drainage of serous effusion may induce and maintain a complete response in some patients with HHV-8-unrelated HIV-negative PEL-like lymphoma, which represents an excellent treatment option for elderly patients with this disease.
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              Pleural effusion in long-term hemodialysis patients.

              As a consequence of the expanded use of long-term hemodialysis and extended life spans, complications of chronic renal failure are encountered with an increased frequency among uremic patients. Such patients may develop many thoracic and extrathoracic problems--most frequently uremic pleuritis and pericarditis, uremic pneumonia, infection, and metastatic pulmonary calcification. We retrospectively analyzed the medical records of 257 patients who had received long-term hemodialysis between 1990 and 2006 to better understand the incidence, causes, and clinical features of pleural effusions in this population. The incidence of pleural effusion in hospitalized patients receiving long-term hemodialysis was 20.2% (n=52; mean age, 55.83 +/- 16.56 years; male-to-female ratio, approximately 3:2). Pleural effusion resulted from hypervolemia in 61.5% and was bilateral in 68.8% of patients. Unilateral effusion was present in 25 of 52 (48%) patients. The most frequent causes of unilateral effusion were hypervolemia (n=9) and parapneumonic effusion (n=5). Thoracenteses were performed in 14 of the 52 patients in the study group. Of thoracenteses performed, 64.3% of the patients had transudative pleural effusion and 35.7% had exudative effusion. Transudative pleural effusion resulted from hypervolemia in 66.7% and heart failure in 22.2%. Of the patients with transudative effusion, 85.7% were bilateral. The most frequent cause of exudative pleural effusion was uremic pleuritis, which occurred in 40% of the patients. The most common symptom was dyspnea, which occurred in 53.8% of patients. In conclusion, pleural effusions are common in patients receiving chronic hemodialysis. Thoracentesis may be performed in patients with unilateral pleural effusion. Since hypervolemia was the most common cause of pleural effusion, this complication should not be considered an obstacle in renal transplant recipients.
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                Author and article information

                Journal
                Case Rep Nephrol Urol
                Case Rep Nephrol Urol
                CRU
                Case Reports in Nephrology and Urology
                S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )
                1664-5510
                May-Aug 2014
                21 May 2014
                21 May 2014
                : 4
                : 2
                : 95-102
                Affiliations
                [1] aDepartment of Internal Medicine, Okinawa Yaeyama Hospital, Okinawa, Japan
                [2] bDepartment of General Medicine, Okinawa Yaeyama Hospital, Okinawa, Japan
                [3] cDepartment of Yonaha Medical Clinic, Okinawa, Japan
                Author notes
                *Yosuke Sasaki, MD, Department of General Medicine and Emergency Care, Toho University School of Medicine, Omori Hospital, 6-11-1, Omori-Nishi, Ota-Ku, Tokyo 143-8541 (Japan), E-Mail pqrstbb@ 123456yahoo.co.jp
                Article
                cru-0004-0095
                10.1159/000363223
                4067712
                da243a59-6de0-4c53-a0da-6d822c988253
                Copyright © 2014 by S. Karger AG, Basel

                This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OA-license), applicable to the online version of the article only. Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

                History
                Page count
                Tables: 2, References: 10, Pages: 8
                Categories
                Published online: May, 2014

                Nephrology
                primary effusion lymphoma,thoracentesis,kaposi's sarcoma herpes virus/human herpes virus 8-negative effusion-based lymphoma,primary effusion lymphoma-like lymphoma,hemodialysis,human herpes virus 8

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