common etiologies of pleural effusion in this patient group are heart failure , volume overload , parapneumonic effusion , tuberculotic pleuritis , and uremic pleuritis . because of the frequency with which this complication is associated with volume overload or heart failure , empirical reduction of the dry weight ( dw ) is often attempted without thoracentesis . primary effusion lymphoma ( pel ) is a large - cell non - hodgkin lymphoma that presents with characteristic lymphomatous effusions in the absence of solid tumor masses and in association with human herpes virus 8 ( hhv8 ) infection in immunocompromised individuals , especially patients with human immunodeficiency virus ( hiv ) infection , and rarely in the elderly . herein , we report an 86-year - old hiv - negative male on hd with pel who presented with refractory left - sided pleural effusion as the first case report of pel developing in a patient receiving hd . an 86-year - old man on hd visited the emergency department ( ed ) of okinawa yaeyama hospital with the complaint of worsening dyspnea . the symptom had started with mild shortness of breath on exertion several months ago and had gradually exacerbated . given the frequency of dyspnea associated with volume overload and/or heart failure in hd patients , his doctor in the hd clinic assumed that the symptom was caused by heart failure associated with volume overload and attempted to empirically reduce his dw . the doctor failed to reduce his dw because of the frequent falling of the blood pressure during hd . furthermore , while his dyspnea became worse , the doctor had to increase his dw by as much as 2 kg over 3 weeks to prevent blood pressure falling and leg cramping during hd . the patient visited our ed with the complaint of an acute worsening of dyspnea during the previous few days . he denied having chest pain , cough , sputum , orthopnea or paroxysmal nocturnal dyspnea , fever , chills , night sweats , or anorexia . he had a past medical history of end - stage renal disease ( esrd ) due to unknown etiology and was on scheduled hd ( 4 h per day at 3 days per week ) for 4 years with an uneventful course . he also had well - controlled essential hypertension and chronic atrial fibrillation which was treated by anticoagulation with warfarin for 4 years . he developed a hemorrhagic gastric ulcer that was probably associated with excessive anticoagulation 1 year ago . he smoked 2 packs of cigarettes a day for 30 years up until 30 years ago . family history was noncontributory . on physical examination , he was alert and appeared slightly sick . his recent dw was 46.0 kg with an increase of 2 kg over 3 weeks . his blood pressure was 130/60 mm hg , pulse was irregularly irregular with a rate of 50 beats per minute , respiration rate was 18 per minute , and body temperature was 37.4c . chest x - ray ( cxr ) showed massive left - sided pleural effusion without findings of pulmonary congestion or masses . ( a cxr performed 7 months previous at the hd clinic showed no pleural effusion . ) blood examination was remarkable for monocytosis ( 17.4% ) , hypoalbuminemia ( 2.5 mg / dl ) , elevated serum thyroid stimulating hormone over 10 g / ml with positive anti - thyroid peroxidase antibody , and positive result for quantiferon - tb2 g testing . the pleural effusion was grossly bloody and exudative with atypical cells , massive mesothelial pleocytosis , and elevation of lactate dehydrogenase ( table 2 ) . both fluorescent stain and ziehl - neelsen stain of the effusion detected no acid - fast bacilli . computed tomography scans with contrast material of the neck , thorax , abdomen , and pelvis detected no findings suggesting a primary lesion of the malignant tumor or lymphadenopathy . given the massive malignant pleural effusion at the unilateral side in the absence of solid tumor masses , we suspected pel of the left thoracic cavity . we also prescribed 25 g per day of levothyroxine for subclinical hypothyroidism due to hashimoto thyroiditis , although the association with pleural effusion is unlikely . with consideration of his age , underlying illness such as esrd , the lack of established treatment for pel , and the request from his family members , we gave up treating him with chemotherapy . as per the family 's wishes , his daughter informed the patient of the diagnosis . we treated the patient with the goal of living at home with his family comfortably . a cxr on the date of discharge revealed that there was no further accumulation of effusion . the patient was referred to our ed 1 month after the previous discharge with the complaint of dyspnea and left pleural effusion . after discontinuation of warfarin and administration of vitamin k , we performed another thoracic tube placement . although he consequently developed re - expansion pulmonary edema ( which presented as dyspnea with bilateral wheeze , crackles , and symmetric pulmonary edema on cxr ) that required noninvasive positive pressure ventilation ( nppv ) and bacteremia with enterobacter cloacae treated with intravenous ceftriaxone ( ctrx ) for 14 days , his symptoms eventually subsided . given the symptomatic accumulation of effusion after less than 1 month , we performed pleurodesis with 5 g of talc to the left thorax . three months following the second admission , he visited the ed due to general fatigue . he was evaluated for anemia ( hemoglobin 7.6 g / dl ) and was transfused with the diagnosis of mixed anemia of chronic disease due to pel and renal anemia . his condition was fully improved with transfusion and rehabilitation and he was discharged after 10 days hospitalization . two weeks following the previous hospitalization , he was referred to the ed due to acute hypoxia following a productive cough for 2 days . we treated him with intravenous ctrx 1 g every 12 h , intravenous ciprofloxacin 200 mg every 12 h , intravenous nitroglycerin , nppv , and emergent hd with the extracorporeal ultrafiltration method for clinical diagnosis of pneumonia combined with acute heart failure . despite our intensive treatment , considering his underlying condition and the pain associated with treatment , we started intravenous morphine for palliation . he died peacefully the day after admission , 7 months after his diagnosis of pel . pel is a large - cell non - hodgkin lymphoma presenting with characteristic lymphomatous effusions in the absence of solid tumor masses . pel was first recognized as a distinct entity in the world health organization 's classification of neoplastic diseases of the hematopoietic and lymphoid tissues in 2001 . it is characteristically associated with hhv8 infection , which has been implicated in the pathogenesis of pel . co - infection with epstein - barr virus ( ebv ) is commonly found , though ebv may not be pathogenic for pel . null lymphocyte phenotype : cd45 is expressed , but common primary pan - b - cell ( cd19 , cd20 , cd79a , surface immunoglobulins ) and t - cell ( cd3 , cd4 , cd8 ) markers are absent . instead , however , markers of lymphocyte activation ( cd30 , cd38 , cd71 , epithelial membrane antigen , human leukocyte antigen - dr ) and plasma cell differentiation ( cd138 ) are often present . definitive diagnosis hinges on the detection of viral infection by hhv8 in the neoplastic cells . at first , pel was considered to be associated with hiv infection because the majority of cases occur in young to middle - aged males , either homosexual or bisexual , with hiv infection . however , it can also occur in hiv - negative individuals who are immunocompromised as a result of solid - organ transplantation or cirrhosis . in rare instances , pel can affect elderly individuals who are otherwise immunocompetent , especially those who live in geographic areas with high hhv8 prevalence , such as the mediterranean region . even in japan , where hhv8 prevalence is not high , several cases of pel in elderly patients have been reported , especially in patients with malignant solid tumors [ 4 , 5 ] . according to these reports , a combination of advanced age and immunocompromised status such as that arising from malignant solid tumors can be regarded as a risk factor of pel . given that it has also been considered that hd results in an immunocompromised status , a combination of advanced age and hd can predispose individuals to pel . so far , this has been the first case report of pel developing in an elderly patient receiving hd . standard chemotherapy regimens for non - hodgkin lymphoma ( e.g. , cyclophosphamide , doxorubicin , vincristine , and prednisolone ) have been attempted with poor outcome ( median survival is less than 6 months ) . our patients survived for 7 months after diagnosis , which seems to be a common clinical outcome for cases of pel . it is important to mention kaposi 's sarcoma herpes virus / hhv8-negative effusion - based lymphoma ( kshv / hhv8-negative ebl ) , also called hhv8-negative pel or pel - like lymphoma , a newly proposed distinct entity . kshv / hhv8-negative ebl is a large b - cell lymphoma with lymphomatous effusions without association with hhv8 infection expressing pan - b - cell markers . this lymphoma is usually seen in fluid overload states and has been reported to have good prognosis when compared to pel . although this lymphoma has been reported as a subtype of pel without association with hhv8 infection because of its clinical and cytomorphological similarity with pel , this entity is distinguished from pel by three points : the lack of association with hhv8 infection , the association with fluid overload status , and the prognosis . kshv / hhv8-negative ebl is characteristically seen in individuals with an underlying medical condition such as cirrhosis or heart failure , and some cases have been effectively treated with pleural aspiration without chemotherapy [ 7 , 8 ] . this good response to pleural effusion eradication may explain the better outcome of kshv / hhv8-negative ebl compared to pel . considering that fluid overload is common in hd patients , making the distinction between pel and kshv / hhv8-negative ebl by evaluating hhv8 infection is very important because the prognosis and etiologies differ . in this case , positive hhv8 immunostaining , resistance to pleurodesis and repeated pleural drainage , and short survival interval support a diagnosis of pel . in addition to common causes of pleural effusion in the general population such as parapneumonic effusion or heart failure , pleural effusion due to fluid overload , tuberculotic pleuritis , and uremic pleuritis are also common in patients receiving hd . tuberculotic pleuritis has been an especially important issue for clinicians because of its public health implications . although it is classically thought of as pleural effusion in patients with esrd without hd , it has recently been reported that uremic pleuritis exists even in patients already receiving hd and is underestimated . a recent study reported that uremic pleuritis comprised 40% of exudative effusion and was the most common etiology of exudative effusion . because uremic pleuritis responds to improvement in hd , this entity is now gathering clinical attention . cases of isolated pleural effusion due to high - output heart failure as a result of a giant vascular access have been reported [ 9 , 10 ] . due to these common , unique , and important etiologies for this patient group , the prevalence and impact of malignant pleural effusion in hd patients may be less than that of the general population . in spite of the fact that thoracentesis and analysis of the pleural effusion is a swift and useful method to distinguish the etiology of effusion in most cases , thoracentesis seems to be infrequently performed in clinical settings . in an article analyzing 257 hospitalized patients with long - term hd , thoracentesis was performed in only 26.9% . even in a report on hd patients admitted for pleural effusion , the reason for the infrequency of thoracentesis is likely due to the fact that most pleural effusion , especially bilateral , is associated with fluid overload , heart failure , or uremic pleuritis and responds to the fluid reduction or improvement of the hd prescription . however , clinicians should be aware that this empiricism involves a risk of overlooking or delaying the diagnosis of potentially fatal etiologies that require specific treatments such as infection or kshv / hhv8-negative ebl . even in incurable malignant effusions , recognizing the diagnosis and prognosis will aid patient care . thus , this case report of pel developing in an elderly patient receiving hd , which is a combination of risk factors of pel , may arouse clinicians attention regarding the importance of evaluation for pleural effusion in hd patients , especially when the effusion or symptoms associated with the pleural effusion are refractory to the reduction of the dw . further studies on pleural effusion in hd patients , pel , and kshv / hhv8-negative ebl are warranted . on behalf of all authors , the corresponding author states that there are no conflicts of interest .
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 .