Humans ; Lymphoma, Primary Effusion ; Lymphoma ; Fatal Outcome

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 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*

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

Primary effusion lymphoma (PEL) is a human herpes virus 8 (HHV8)-positive large B-cell neoplasm that presents as an effusion with no detectable tumor in individuals with human immunodeficiency virus infection or other immune deficiencies. PEL is an aggressive neoplasm with a poor prognosis. PEL cells show diverse morphologies, ranging from immunoblastic or plasmablastic to anaplastic. The immunophenotype of PEL is distinct, but its lineage can be misdiagnosed if not assessed thoroughly. PEL cells usually express CD45, lack B- and T-cell-associated antigens, and characteristically express lymphocyte activation antigens and plasma cell-associated antigens. Diagnosis of PEL often requires the demonstration of a B-cell genotype. HHV8 must be detected in cells to diagnose PEL. In most cases, PEL cells also harbor the Epstein-Barr virus (EBV) genome. Similar conditions associated with HHV8 but not effusion-based are called "extracavitary PELs." PELs should be differentiated from HHV8-negative, EBV-positive, body cavity-based lymphomas in patients with long-standing chronic inflammation; the latter can occur in tuberculous pleuritis, artificial pneumothorax, chronic liver disease and various other conditions. Despite their morphological similarity, these various lymphomas require different therapeutic strategies and have different prognostic implications. Correct diagnosis is essential to manage and predict the outcome of patients with PEL and related disorders.
B-Lymphocytes ; Diagnosis ; Genome ; Genotype ; Herpesvirus 4, Human ; HIV ; Humans ; Inflammation ; Liver Diseases ; Lymphocyte Activation ; Lymphoma* ; Lymphoma, Primary Effusion* ; Plasma ; Pleurisy ; Pneumothorax, Artificial ; Prognosis

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

Human herpes virus-8 (HHV-8)-negative primary effusion lymphoma (PEL) is frequently observed in patients with chronic liver disease. We describe herein a case of PEL in a patient with chronic kidney disease. A 59-year-old woman presented with dyspnea. The patient had a history of uncontrolled pleural effusion. The pleural effusion revealed a malignant cell-dominant exudate. Serological tests were negative for human immunodeficiency virus (HIV). Initial chest and abdomen computed tomography showed bilateral pleural effusion; however, no evidence of a tumor mass or lymph node enlargement was found. A malignant lymphoma of diffuse large B-cell type was confirmed by pleural fluid cytology. Immunohistochemical staining of malignant cells was negative for HHV-8. She was treated with rituximab and CHOP (cyclophosphamide, adriamycin, vincristine, prednisolone) chemotherapy. We report a case of PEL in a patient with chronic kidney disease that may be a plausible predisposing factor for HHV-8-negative PEL.
Abdomen ; Antibodies, Monoclonal, Murine-Derived ; B-Lymphocytes ; Doxorubicin ; Dyspnea ; Exudates and Transudates ; Female ; Herpesvirus 8, Human ; HIV ; Humans ; Liver Diseases ; Lymph Nodes ; Lymphoma ; Lymphoma, Primary Effusion ; Middle Aged ; Pleural Effusion ; Renal Insufficiency, Chronic ; Serologic Tests ; Thorax ; Vincristine ; Rituximab

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

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