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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 references 12

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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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            Diseases and drug-related interventions affecting host defence.

             S. de Marie (1993)
            Recognition of an impaired immune system has implications for the diagnosis, treatment and outcome of infections. Not only the type of immunological dysfunction but also the degree of the dysfunction is important. A review is presented of the systemic diseases and immunosuppressive agents which may result in defects in host defence and associated infections. Especially in transplant patients and patients with HIV infection, knowledge of the stage of the host defense defect is important for the success of prophylactic regimens and for the clinical approach in the event of infection.
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              Pleural effusion in long-term hemodialysis patients.

               S Sezer,  T Bakirci,  S Akçay (2007)
              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
                CRU
                CND
                10.1159/issn.2296-9705
                Case Reports in Nephrology and Dialysis
                S. Karger AG
                2296-9705
                2014
                May – August 2014
                21 May 2014
                : 4
                : 2
                : 95-102
                Affiliations
                Departments of aInternal Medicine and bGeneral Medicine, Okinawa Yaeyama Hospital, and cYonaha 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@yahoo.co.jp
                Article
                363223 PMC4067712 Case Rep Nephrol Urol 2014;4:95-102
                10.1159/000363223
                PMC4067712
                24987405
                © 2014 S. Karger AG, Basel

                Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) ( http://www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                Page count
                Tables: 2, Pages: 8
                Categories
                Published: May 2014

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