Humans ; Lymphoma, Primary Effusion ; Lymphoma ; Fatal Outcome

A primary effusion lymphoma is a rare type of non-Hodgkin's lymphoma where serous cavities are involved. That-cause peritoneal, pleural and pericardial effusions without any lymphadenopathy. They affect immunosuppressive patients with human herpes virus-8 being the suspected etiological agent. The prognosis is usually poor despite treatment. Herein, the case of an immunocompetent patient with ascites and pleural effusion diagnosed as primary effusion lymphoma is presented and discuss the case in the light of the current literature.
Ascites/*diagnosis ; Fatal Outcome ; Humans ; Lymphoma, Primary Effusion/*diagnosis ; Male ; Middle Aged ; Pleural Effusion/*diagnosis

PURPOSE: Primary effusion lymphoma (PEL) is a type of body cavity–based lymphoma (BCBL). Most patients with PEL are severely immunocompromised and seropositive for human immunodeficiency virus (HIV). We investigated the distinctive clinicopathologic characteristics of BCBL in a country with low HIV burden. MATERIALS AND METHODS: We retrospectively collected data on the clinicopathologic characteristics, treatments, and outcomes of 17 consecutive patients with BCBL at nine institutions in Korea. RESULTS: Latency-associated nuclear antigen 1 (LANA1) immunostaining indicated that six patients had PEL, six patients had human herpesvirus 8 (HHV8)-unrelated BCBL, and five patients had HHV8-unknown BCBL. The patients with PEL exhibited no evidence of immunodeficiency except for one who was HIV positive. One (20%) and four (80%) patients with PEL and six (100%) and zero (0%) patients with HHV8-unrelated BCBL were positive for CD20 and CD30 expression, respectively. The two patients with PEL (one HIV-positive and one HIV-negative patient) with the lowest proliferation activity as assessed by the Ki-67 labeling index survived for > 1 and > 4 years without chemotherapy, respectively, in contrast to the PEL cases in the literature, which mostly showed a high proliferation index and poor survival. CONCLUSION: PEL mostly occurred in ostensibly immunocompetent individuals and had a favorable outcome in Korea. A watchful waiting approach may be applicable for managing HIV-seronegative patients with PEL with a low Ki-67 labeling index. A possible trend was detected among LANA1, CD20, and CD30 expression in BCBL.
Drug Therapy ; Herpesvirus 8, Human ; HIV ; Humans ; Korea ; Lymphoma ; Lymphoma, Primary Effusion ; Prevalence ; Retrospective Studies ; Watchful Waiting

Primary effusion lymphoma (PEL) is a recently described rare type of non-Hodgkin's lymphoma which develops in serous body cavity without clinically identifiable tumor masses. Human herpesvirus (HHV)-8 was recognized to play a pathogenic role in the development of PEL in immunocompromised patients such as human immunodeficiency virus (HIV) positive patients or elderly. However, several cases of HHV-8 negative PEL have been reported. Here we describe a case of HHV-8 positive PEL with both of hepatocellular carcinoma and liver cirrhosis and two cases of HHV-8 negative PEL with liver cirrhosis and fatty liver each. Considering the reported and current HHV-8 negative PEL cases, we need to expand the concept of PEL.
Aged ; Carcinoma, Hepatocellular ; Fatty Liver ; Herpesvirus 8, Human ; HIV ; Humans ; Immunocompromised Host ; Liver Cirrhosis ; Lymphoma, Non-Hodgkin ; Lymphoma, Primary Effusion*

Primary effusion lymphoma (PEL) is a rare type of non-Hodgkin’s lymphoma arising from a B-cell lineage characterized by the formation of malignant effusion in body cavities without evidence of a detectable tumor. The effusion contains tumor cells universally infected with human herpesvirus 8 (HHV8), which is the critical factor differentiating PEL from HHV8-unrelated PEL-like lymphoma (PEL-LL). This report describes a 77-year-old male patient with pleural effusion and ascites, containing lymphoma cells expressing a B-cell phenotype, but without markers of HHV8 in immunocytochemical analysis. The patient was diagnosed with PEL-LL and treated with six cycles of rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP), which resulted in a complete remission. The patient is currently disease-free 15 months post-treatment. To the best of our knowledge, this is the first report on administration of R-CHOP in a PEL-LL patient in South Korea.
Aged* ; Ascites ; B-Lymphocytes ; Cyclophosphamide* ; Doxorubicin* ; Herpesvirus 8, Human ; Humans* ; Korea ; Lymphoma* ; Lymphoma, Primary Effusion ; Male ; Phenotype ; Pleural Effusion ; Prednisolone* ; Rituximab* ; Vincristine*

Primary effusion lymphoma (PEL) is a rare type of lymphoma that arises in the body cavity without detectable masses. It is associated with human herpes virus-8 (HHV-8), Epstein-Barr virus (EBV), and human immunodeficiency virus (HIV). Recently, PEL unrelated to viral infection has been reported and it has been termed HHV-8 unrelated primary effusion lymphoma-like lymphoma (HHV-8 unrelated PEL-like lymphoma). Here, we report a case of HHV-8 unrelated PEL-like lymphoma in an 80-year-old woman. Chest X-ray and computed tomography revealed left-sided pleural effusion. Pleural effusion analysis and mediastinoscopic biopsy showed atypical cells that had originated from the B cells. The cells were positive for CD20 and bcl-2, but negative for CD3, CD5, CD21, CD30, CD138, epithelial membrane antigen, and HHV-8. Serological tests for HIV and EBV were negative. Considering the patient's age, further treatments were not performed. She has shown good prognosis without chemotherapy for more than 18 months.
B-Lymphocytes ; Biopsy ; Female ; Herpesvirus 4, Human ; Herpesvirus 8, Human ; HIV ; Humans ; Lymphoma ; Lymphoma, Primary Effusion ; Mucin-1 ; Pleural Effusion ; Prognosis ; Serologic Tests ; Thorax

Primary effusion lymphoma (PEL) is a distinct clinical and pathologic entity characterized by malignant lymphomatous effusion in body cavities, without a detectable tumor mass. The tumor is usually associated with human herpes virus 8 (HHV-8) infection and occurs principally in immunodeficient patients, most commonly those with human immunodeficiency virus (HIV) infection. We report a rare case of PEL in an elderly patient negative for HHV8 and HIV. The patient had a history of alcoholic liver cirrhosis and presented with abdominal distention. The patient was treated with three courses of chemotherapy. In spite of the generally poor prognosis associated with this disease, our patient is still alive with no malignant lymphomatous cells in the peritoneal cavity 24 months after treatment.
Aged ; Alcoholics ; Ascites ; HIV ; Humans ; Liver Cirrhosis ; Liver Cirrhosis, Alcoholic ; Lymphoma ; Lymphoma, Non-Hodgkin ; Lymphoma, Primary Effusion ; Peritoneal Cavity ; Prognosis ; Viruses

A 39-year-old man infected with human immunodeficiency virus (HIV) was admitted to our hospital because of sudden onset of chest pain. Chest radiography revealed pneumothorax of the right lung. Computed tomographic scans disclosed a 5.8-cm-sized emphysematous bulla in the right middle lobe of the lung. Histologically, the wedge-resected lung showed medium to large atypical cells within the bullous cavity of the Pneumocystis jirovecii pneumonia, without solid mass formation. These atypical cells were confirmed to be large B-cell lymphoma, Epstein-Barr virus-positive and human herpesvirus 8-negative. Therefore, this case was not diagnosed as primary effusion lymphoma, but effusion-based lymphoma arising in an emphysematous cavity of an HIV-infected patient. This type of effusion-based lymphoma has never been reported, and, although rare, it should be noted in order to clinically diagnose this lymphoma.
Adult ; Chest Pain ; Herpesvirus 4, Human ; Herpesvirus 8, Human ; HIV* ; Humans* ; Lung ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Primary Effusion ; Pneumocystis jirovecii ; Pneumonia ; Pneumothorax ; Radiography ; Thorax

Pyothorax-associated lymphoma is a distinct type of malignant lymphoma composed of lymphocytes, mostly of a B-cell nature. It occurs in patients with a long history of pyothorax and is strongly associated with Epstein-Barr virus infection. We experienced a case of pyothorax-associated lymphoma in a 66-year-old male. The patient had chronic pyothorax after tuberculous pleurisy was treated about 37 years earlier and was hospitalized for a recently occurring chest wall mass. A core needle biopsy of the chest wall mass led to a diagnosis of diffuse large B-cell lymphoma. Pyothorax-associated lymphoma is rare in Korea, but should be considered in patients with chronic pyothorax and a recently occurring pleural mass.
Aged ; B-Lymphocytes ; Biopsy, Large-Core Needle ; Empyema ; Empyema, Pleural ; Herpesvirus 4, Human ; Humans ; Korea ; Lymphocytes ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Primary Effusion ; Male ; Thoracic Wall ; Tuberculosis, Pleural

Pyothorax-associated lymphoma is a distinct type of malignant lymphoma composed of lymphocytes, mostly of a B-cell nature. It occurs in patients with a long history of pyothorax and is strongly associated with Epstein-Barr virus infection. We experienced a case of pyothorax-associated lymphoma in a 66-year-old male. The patient had chronic pyothorax after tuberculous pleurisy was treated about 37 years earlier and was hospitalized for a recently occurring chest wall mass. A core needle biopsy of the chest wall mass led to a diagnosis of diffuse large B-cell lymphoma. Pyothorax-associated lymphoma is rare in Korea, but should be considered in patients with chronic pyothorax and a recently occurring pleural mass.
Aged ; B-Lymphocytes ; Biopsy, Large-Core Needle ; Empyema ; Empyema, Pleural ; Herpesvirus 4, Human ; Humans ; Korea ; Lymphocytes ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Primary Effusion ; Male ; Thoracic Wall ; Tuberculosis, Pleural