ABSTRACT
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.
Subject(s)
Humans , Drug Therapy , Herpesvirus 8, Human , HIV , Korea , Lymphoma , Lymphoma, Primary Effusion , Prevalence , Retrospective Studies , Watchful WaitingABSTRACT
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.
Subject(s)
Aged , Humans , Male , Ascites , B-Lymphocytes , Cyclophosphamide , Doxorubicin , Herpesvirus 8, Human , Korea , Lymphoma , Lymphoma, Primary Effusion , Phenotype , Pleural Effusion , Prednisolone , Rituximab , VincristineABSTRACT
Plasmacytosis is a common finding in lymph node biopsies and can be seen in diverse circumstances ranging from reactive lymphadenopathy to malignant lymphoma. Familiarity with various histopathologic features of the different entities and awareness of their typical clinical and ancillary study findings are essential for an accurate diagnosis. In this review, we present common and representative nonneoplastic entities and lymphomas that have plasmacytic differentiation or associated plasmacytosis. We focus on the histological classification with an emphasis on the diagnostic approach and areas of diagnostic challenge.
ABSTRACT
We present a case of extracavitary primary effusion lymphoma presenting, as jejunal polyps in a 38-year-old man. This is the first report of this entity from India. Although rare in our country, the diagnosis should be suspected in cases of CD20 negative large cell lymphoma with plasmablastic or immunoblastic differentiation in seropositive patients. Immunostaining for latency-associated nuclear antigen-1 and in situ hybridization for Epstein-Barr virus-associated RNA will confirm the diagnosis.
ABSTRACT
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.
Subject(s)
Female , Humans , Middle Aged , Abdomen , Antibodies, Monoclonal, Murine-Derived , B-Lymphocytes , Doxorubicin , Dyspnea , Exudates and Transudates , Herpesvirus 8, Human , HIV , Liver Diseases , Lymph Nodes , Lymphoma , Lymphoma, Primary Effusion , Pleural Effusion , Renal Insufficiency, Chronic , Serologic Tests , Thorax , Vincristine , RituximabABSTRACT
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.
Subject(s)
Aged , Humans , Male , B-Lymphocytes , Biopsy, Large-Core Needle , Empyema , Empyema, Pleural , Herpesvirus 4, Human , Korea , Lymphocytes , Lymphoma , Lymphoma, B-Cell , Lymphoma, Primary Effusion , Thoracic Wall , Tuberculosis, PleuralABSTRACT
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.
Subject(s)
Aged , Humans , Male , B-Lymphocytes , Biopsy, Large-Core Needle , Empyema , Empyema, Pleural , Herpesvirus 4, Human , Korea , Lymphocytes , Lymphoma , Lymphoma, B-Cell , Lymphoma, Primary Effusion , Thoracic Wall , Tuberculosis, PleuralABSTRACT
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.
Subject(s)
Humans , Male , Middle Aged , Ascites/diagnosis , Fatal Outcome , Lymphoma, Primary Effusion/diagnosis , Pleural Effusion/diagnosisABSTRACT
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.
Subject(s)
Aged , Female , Humans , Abdomen , Adenosine Deaminase , Biopsy , DNA , Dyspnea , Exudates and Transudates , Herpesvirus 8, Human , HIV , Lung , Lymph Nodes , Lymphoma, B-Cell , Lymphoma, Primary Effusion , Physical Examination , Pleura , Pleural Effusion , Pleurisy , Pleurodesis , Polymerase Chain Reaction , Serologic Tests , Thoracic Surgery, Video-Assisted , ThoraxABSTRACT
s Kaposi's sarcoma-associated herpesvirus (KSHV) was first identified as the etiologic agent of Kaposi's sarcoma (KS) in 1994.KSHV infection is necessary,but not sufficient for the development of Kaposi sarcoma (KS),primary effusion lymphoma (PEL),and multicentric Castleman disease (MCD).Advances in the prevention and treatment of KSHV-associated Diseases have been achieved,even though current treatment options are ineffective,or toxic to many affected persons.The identification of new targets for potential future therapies and the randomized trial to evaluate the efficacy of new antivirals are required.
ABSTRACT
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.
Subject(s)
Aged , Humans , Carcinoma, Hepatocellular , Fatty Liver , Herpesvirus 8, Human , HIV , Immunocompromised Host , Liver Cirrhosis , Lymphoma, Non-Hodgkin , Lymphoma, Primary EffusionABSTRACT
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.