Body Cavity–Based Lymphoma in a Country with Low Human Immunodeficiency Virus Prevalence: A Series of 17 Cases from the Consortium for Improving Survival of Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

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.

Human Herpesvirus 8–Unrelated Primary Effusion Lymphoma–Like Lymphoma in an Elderly Korean Patient with a Good Response to Rituximab Plus Cyclophosphamide, Doxorubicin, Vincristine, and Prednisolone / Journal of the Korean Cancer Association, 대한암학회지

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.

Human Herpesvirus 8-Negative and Epstein-Barr Virus-Positive Effusion-Based Lymphoma in a Patient with Human Immunodeficiency Virus

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.

Current Concepts in Primary Effusion Lymphoma and Other Effusion-Based Lymphomas

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.

VIH y Linfoma, una reflexión necesaria: reposte de serie de casos / HIV and LIMPHOMA, a necessary reflection: Case Series Report

La relación entre el deterioro de la función inmunológica y el desarrollo de linfoma es bien conocido en los pacientes con algún grado de inmunosupresión, en los que se observan linfomas con mayor frecuencia que en la población en general, por lo tanto no es sorprendente la asociación entre el virus de inmunodeficiencia humana (VIH) y el desarrollo de este tipo de neoplasia, con - siderándose como definitoria de enferme- dad avanzada. Los linfomas no Hodgkin (LNH) constituyen la segunda neoplasia más frecuente asociada a la infección por VIH, identificándose dos formas anatomo- clínicas principales: Los LNH Sistémicos y los Linfomas primarios del sistema nervioso central (LPSNC), estos últimos se han reducido considerablemente desde la intro - ducción del tratamiento antirretroviral de gran actividad (TARGA). Aunque no se considera definitorio de enfer - medad avanzada, otras neoplasias relacio - nadas con VIH son Enfermedad de Hodgkin y leucemias. Se presenta a continuación una serie de 4 casos clínicos de pacientes con VIH, atendidos en el Centro de Atención integral pediátrico/ Hospital Nacional Doctor Mario Catarino Rivas (HNMCR), en el año 2013, donde se puede observar la variedad de presentación de los linfomas y su relación con el estado inmunológico y virológico...(AU)

A Case of Human Herpes Virus-8 Unrelated Primary Effusion Lymphoma-Like Lymphoma Presented as Pleural Effusion / 결핵및호흡기질환

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.

A Case of Primary Effusion Lymphoma in a Patient with Chronic Kidney Disease / 대한내과학회지

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.

A Case of Chronic Pyothorax-Associated Lymphoma / 대한내과학회지

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.

A Case of Chronic Pyothorax-Associated Lymphoma / 대한내과학회지

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.

Primary Effusion Lymphoma: An Untrivial Differential Diagnosis for Ascites

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.

A case of primary effusion lymphoma / 대한내과학회지

A 78-year-old woman was referred to this hospital because of exertional dyspnea for 2 weeks. Although she was treated with anti-tuberculous medication under the assumption that she had tuberculous pleuritis, her dyspnea continued. Physical examination revealed diminished breath sounds over the left lung field. Chest radiography showed a large left pleural effusion. The pleural effusion revealed malignant cell dominant exudates with a high adenosine deaminase level. Serological tests were negative for HIV. Computed tomography scan of the chest and abdomen showed pleural effusion, but no mass or pathological lymph nodes were detected. The biopsy specimen of the pleura was diagnosed as malignant B cell lymphoma with plasmacytoid features, suggesting a possibility of primary effusion lymphoma. Polymerase chain reaction assay of tumor was positive for HHV-8 DNA. She underwent pleurodesis during Video-Assisted Thoracic Surgery, and her dyspnea subsequently improved.

A case of primary effusion lymphoma in a patient with alcoholic liver cirrhosis / 대한내과학회지

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.

Three Cases of Primary Effusion Lymphoma / 대한혈액학회지

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.

A case of non-Hodgkin's lymphoma presented only as unilateral pleural effusion / 대한내과학회지

We experienced a case of non-Hodgkin's lymphoma presented only as right side pleural effusion, that is primary effusion lymphoma (PEL), in a 75 year-old male patient in Korea where is the endemic area of tuberculosis. He visited our hospital complaining of exertional dyspnea. He did not have B symptoms. The breathing sound was decreased on the right side chest, but we could not find external lymphadenopathy or hepatosplenomegaly on physical examination. Simple chest radiograph showed right side pleural effusion. The cells of pleural fluid were lymphocyte-predominant and the pH, protein, lactate dehydrogenase, adenosine deaminase of the fluid was 7.31, 38 g/L, 381 U/L, 31 U/L, respectively. The biopsy specimen of the parietal pleura was diagnosed as non-Hodgkin's lymphoma of small lymphocytic type. Computed tomograph of the chest, abdomen and pelvis, and the biopsy of bone marrow were negative for disease. We tried up to 3 cycles of chemotherapy with adriamycin, vincristine, cyclophosphamide and prednisolone and there was a marked decrease in the amount of the pleural effusion.

A Case of Primary Pleural Effusion Lymphoma / 대한혈액학회지

Primary effusion lymphoma (PEL) is a recently described subtype of malignant lymphoma which develops in association with human herpesvirus-8 (HHV-8) in human immunodeficiency virus (HIV)-infected patients. PEL presents predominantly as lymphomatous effusion in the body cavities without a definite mass or lymphadenopathy. The malignant cell has large-cell morphology with null immunophenotype and B-cell genotype. We describe an unusual case of HIV negative HHV-8 negative PEL patient. A 48-year-old man presented with symptoms of right lower chest pain and fatigue for 3 months. Chest radiography and CT scan showed right pleural effusion and pleural thickening and no evidence of lymphadenopathy. Examination of the pleural fluid revealed lympho-dominant exudate and the results of all cultures were negative. A malignant lymphoma of diffuse large B-cell type was confirmed by pleural biopsy. The results of serologic studies for HIV and PCR of HHV- 8 using pleura fluid and tissue were all negative.

PCR Study for Human Herpesvirus 8 in Korean Patients with Several Cutaneous Diseases / 대한피부과학회지

BACKGROUND: The association of Kaposi's sarcoma-associated human herpesvirus, or human herpesvirus 8 (HHV8) with various skin diseases has been described in many reports. However, only 3 human diseases: Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman disease were proved to be associated with HHV8. In addition to Karposi's sarcoma, sarcoidosis and Kikuchi's disease were also described to be associated with HHV8. OBJECTIVE: The purpose of this study was aimed at the association of HHV8 with various skin diseases, such as Kaposi's sarcoma, sarcoidosis, and Kikuchi's disease. METHODS: We performed PCR to examine whether the 233-bp segment of the viral DNA of HHV8 was detected in Korean patients with Kaposi's sarcoma, sarcoidosis, Kikuchi's disease, angiosarcoma, and angiolymphoid hyperplasia with eosinophilia. The medical records and histopathological specimens of patients diagnosed at Asan Medical Center from 1989 to 1999 were reviewed. RESULTS: HHV8 DNA sequences were identified in 9 (69%) of sarcoid tissues from 12 patients with sarcoidosis and in all (100%) of from 8 patients with Kaposi's sarcoma but not in 2 tissues from 2 patients with Kikuchi's disease, in 3 tissues from 2 patients with angiosarcoma and 2 tissues from 2 patients with angiolymphoid hyperplasia with eosinophilia. CONCLUSION: HHV8 may be strongly associated with the pathogenesis of Kaposi's sarcoma and sarcoidosis. However, the association with Kikuchi's disease, angiosarcoma, and angiolymphoid hyperplasia with eosinophilia was denied in this study. Further extensive study will be needed.