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

B cell lymphoma and Kaposi's sarcoma are the most common tumors in AIDS patients. They show more aggressive behavior and poor prognosis in AIDS patients than in the general population. Lymphoma and Kaposi's sarcoma in AIDS patients are associated with Epstein-Barr virus and human herpesvirus 8, respectively. We reported herein a case of lymphoma and Kaposi's sarcoma in an AIDS patient. He presented with a mass on the tongue base and a red papule on the posterior wall of the oropharynx. On the basis of his- tologic findings, immunohistochemical study, and PCR, his illnesses were diagnosed as B cell lymphoma, combined with early Kaposi's sarcoma. Unfortunately, he did not receive any treatment and died of malnutrition 1 month later. To the best of our knowledge, this is the first report of coexisting lymphoma and Kaposi's sarcoma from an AIDS patient in Korea.
B-Lymphocytes* ; Herpesvirus 4, Human ; Herpesvirus 8, Human ; Humans ; Korea ; Lymphoma ; Lymphoma, B-Cell* ; Malnutrition ; Oropharynx ; Polymerase Chain Reaction ; Prognosis ; Sarcoma, Kaposi* ; Tongue

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

A putative gamma herpesvirus, termed human herpesvirus 8 (HHV-8), discovered in recent years, has been implicated as a possible etiologic agent for Kaposi`s sarcoma (KS). In South Korea, the incidence of KS in HIV seropositive individuals is very low. The cause of its rarity as compared with other countries is unclear. The objective of this study was performed to determine the prevalence of infection with HHV-8 and to clarify the cause of low incidence of KS in Korean populations including HIV seropositive individuals. The study population was composed of 200 blood donors, 220 voluntary visitors for sexual transmitted infection (STI)-testing in the public health centers, and 214 HIV-seropositive individuals. For the detection of HHV-8 antibodies, all blood samples were tested using Advanced Biotechnologies Inc`s enzyme-linked immunosorbent assay (ELISA) kits and the reactive samples were retested using Biotrin International SARL`s immunofluorescent assay (IFA). Also, we investigated the seroprevalence of Cytomegalovirus (CMV), Varicella-Zoster virus (VZV) and Epstein-Barr Virus (EBV) in order to get more information of HHV-8 and other human herpesviruses transmission in Korea. The prevalence of specific IgG to HHV-8 among HIV seropositive individuals was 7.0% {95% confidential interval: 4.0-11.3%}. The specific antibody to HHV-8 could be detected only in HIV seropositive men. The prevalences of antibodies to other human herpesviruses unlike HHV-8 were very high even in blood donors. These observations strongly suggest that the rarity of KS in this country may be caused by very low prevalence of HHV-8.
Antibodies ; Biotechnology ; Blood Donors ; Cytomegalovirus ; Enzyme-Linked Immunosorbent Assay ; Herpesviridae ; Herpesvirus 3, Human ; Herpesvirus 4, Human ; Herpesvirus 8, Human* ; HIV ; Humans* ; Immunoglobulin G ; Incidence ; Korea* ; Male ; Prevalence ; Public Health ; Sarcoma* ; Sarcoma, Kaposi ; Seroepidemiologic Studies*

PURPOSE: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis (HNL), is a self-limited disease characterized by cervical lymphadenopathy and fever. The etiology of KFD remains unknown; however, the self-limiting nature of HNL suggests the cause of this disease could be viral infection. For this reason, several viruses have been evaluated as possible etiologies of HNL, including Epstein-Barr virus (EBV), human herpesvirus 6 (HHV6), human herpesvirus 8 (HHV8), and cytomegalovirus (CMV). The aim of this study was to examine the relationship of EBV and HHV6 to HNL. METHODS: Data pertaining to 51 cases with biopsy-confirmed HNL were collected between January 1999 and December 2005, from the Department of Pathology, College of Medicine, Pusan National University, Busan, Korea. The clinical records-including data regarding age, gender, duration of fever, and lymph node involvementwere reviewed retrospectively. The in situ hybridization (ISH) assay was performed by EBER PNA probe (Dako, Capinteria, CA, USA), and immunohistochemistry testing was performed with anti-HHV type 6 monoclonal antibodies (Chemicon, Temecula, CA, USA). RESULTS: The HNL patients in this study were 24 males and 27 females, ranging in age from seven to 61 years (median: 25.9). ISH for EBV was positive in 8/51 (15.7%) biopsies, and immunohistochemistry for HHV6 was positive in 15/51 (29.4%) biopsies. Serologic analysis of EBV IgM was performed in 23 cases; only one patient was positive for EBV IgM and EBV ISH. CONCLUSION: Our study could not provide supportive evidence of a viral pathogenesis for HNL; therefore, cases of HNL may not have a dominant viral cause. However, some rare exceptional cases may have been caused by viral infection.
Antibodies, Monoclonal ; Biopsy ; Cytomegalovirus ; Female ; Fever ; Herpesvirus 4, Human ; Herpesvirus 6, Human ; Herpesvirus 8, Human ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Immunoglobulin M ; Immunohistochemistry ; In Situ Hybridization ; Korea ; Lymph Nodes ; Lymphatic Diseases ; Male ; Retrospective Studies

PURPOSE: Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis (HNL), is a self-limited disease characterized by cervical lymphadenopathy and fever. The etiology of KFD remains unknown; however, the self-limiting nature of HNL suggests the cause of this disease could be viral infection. For this reason, several viruses have been evaluated as possible etiologies of HNL, including Epstein-Barr virus (EBV), human herpesvirus 6 (HHV6), human herpesvirus 8 (HHV8), and cytomegalovirus (CMV). The aim of this study was to examine the relationship of EBV and HHV6 to HNL. METHODS: Data pertaining to 51 cases with biopsy-confirmed HNL were collected between January 1999 and December 2005, from the Department of Pathology, College of Medicine, Pusan National University, Busan, Korea. The clinical records-including data regarding age, gender, duration of fever, and lymph node involvementwere reviewed retrospectively. The in situ hybridization (ISH) assay was performed by EBER PNA probe (Dako, Capinteria, CA, USA), and immunohistochemistry testing was performed with anti-HHV type 6 monoclonal antibodies (Chemicon, Temecula, CA, USA). RESULTS: The HNL patients in this study were 24 males and 27 females, ranging in age from seven to 61 years (median: 25.9). ISH for EBV was positive in 8/51 (15.7%) biopsies, and immunohistochemistry for HHV6 was positive in 15/51 (29.4%) biopsies. Serologic analysis of EBV IgM was performed in 23 cases; only one patient was positive for EBV IgM and EBV ISH. CONCLUSION: Our study could not provide supportive evidence of a viral pathogenesis for HNL; therefore, cases of HNL may not have a dominant viral cause. However, some rare exceptional cases may have been caused by viral infection.
Antibodies, Monoclonal ; Biopsy ; Cytomegalovirus ; Female ; Fever ; Herpesvirus 4, Human ; Herpesvirus 6, Human ; Herpesvirus 8, Human ; Histiocytic Necrotizing Lymphadenitis ; Humans ; Immunoglobulin M ; Immunohistochemistry ; In Situ Hybridization ; Korea ; Lymph Nodes ; Lymphatic Diseases ; Male ; Retrospective Studies

Tegument is the unique structure of a herpesvirion which occupies the space between nucleocapsid and envelope. Accumulating data have indicated that interactions among tegument proteins play a key role in virion morphogenesis. Morphogenesis of gammaherpesviruses including Kaposi's sarcoma-associated herpesvirus (KSHV) and Epstein-Barr virus (EBV) is poorly understood due to the lack of efficient de novo lytic replication in cell culture. Murine gammaherpesvirus-68 (MHV-68) is genetically related to these two human herpesviruses and serves as an effective model to study the lytic replication of gammaherpesviruses. We previously showed that ORF33 of MHV-68 encodes a tegument protein and plays an essential role in virion maturation in the cytoplasm. However, the molecular mechanism of how ORF33 participates in virion morphogenesis has not been elucidated. In this study we demonstrated that ORF38 of MHV-68 is also a tegument protein and is localized to cytoplasmic compartments during both transient transfection and viral infection. Immuno-gold labeling assay showed that ORF38 is only present on virions that have entered the cytoplasmic vesicles, indicating that ORF38 is packaged into virions during secondary envelopment. We further showed that ORF38 co-localizes with ORF33 during viral infection; therefore, the interaction between ORF38 and ORF33 is conserved among herpesviruses. Notably, we found that although ORF33 by itself is distributed in both the nucleus and the cytoplasm, in the presence of ORF38, ORF33 is co-localized to trans-Golgi network (TGN), a site where secondary envelopment takes place.
Animals ; DNA Replication ; genetics ; Gammaherpesvirinae ; genetics ; pathogenicity ; Humans ; Mice ; Viral Envelope Proteins ; genetics ; Viral Matrix Proteins ; genetics ; Virion ; genetics ; Virus Replication ; trans-Golgi Network ; genetics

OBJECTIVE: To investigate the cytokine/chemokine profile in patients with Epstein-Barr virus (EBV)-related hemophagocytic lymphohistiocytosis (HLH), and assess the prognostic value of survival. METHODS: Serum levels of thirty-eight cytokines/chemokines were measured by multiple cytokine assay kit in EBV-related HLH patients, EBV-infected patients, and controls. The expression profile of cytokines/chemokines was compared among groups. The changes of cytokine/chemokine expression in active and remission stage of EBV-related HLH patients were also compared, and the prognostic values for survival were evaluated. RESULTS: Serum levels of interferon-α2 (IFN-α2), interleukin (IL)-6, and IL-7 in EBV-related HLH patients were 33.67(23.23-68.78) pg/ml, (74.95±25.53) pg/ml, and 35.35(19.50-63.55) pg/ml, respectively, which were significantly higher than those in EBV-infected patients［IFN-α2: 16.07(9.87-29.63); IL-6: 55.91±20.29; IL-7: 20.40(13.35-31.40)］ and controls ［IFN-α2: 11.02(4.67-21.25); IL-6:42.64±13.41; IL-7: 16.95(14.95-33.78)］(all P<0.05). Serum levels of IL-8, IL-9, and marcophage-derived chemokine (MDC) in EBV-related HLH patients were 11.00(7.50-15.27) pg/ml, 81.30(40.79-111.0) pg/ml, and (512.6±128.7) pg/ml, respectively, which were significantly higher than those in controls ［IL-8: 6.80(5.56-8.38); IL-9: 41.30(29.82-67.91); MDC: 384.1±156.6］(all P<0.05), but there was no remarkable differences compared with EBV-infected patients (P>0.05). Serum IFN-α2, IL-6, IL-7, IL-8, IL-9, and MDC in survival and death groups of EBV-related HLH patients were analyzed by receiver operating characteristic curve with area under curve of 0.781, 0.778, 0.633, 0.805, 0.562, and 0.657, respectively (P=0.019, 0.021, 0.269, 0.015, 0.607, and 0.190). IFN-α2, IL-6, and IL-8 had good predictive effect on survival. Serum level of IFN-α2, IL-6, and MDC of EBV-related HLH patients in remission stage were significantly lower than those in active stage (P<0.05), while IL-7, IL-8, and IL-9 were not different (P>0.05). CONCLUSION IFN-α2, IL-6, IL-7, IL-8, IL-9, and MDC may take part in the pathogenesis of EBV-related HLH.
Humans ; Lymphohistiocytosis, Hemophagocytic/complications* ; Herpesvirus 4, Human ; Cytokines/metabolism* ; Epstein-Barr Virus Infections/complications* ; Interleukin-6 ; Clinical Relevance ; Interleukin-7 ; Interleukin-8 ; Interleukin-9 ; Chemokines ; Interferons

BACKGROUND: Angioimmunoblastic T-cell lymphoma (AITL) is a complex lymphoproliferative disorder and often mimics a viral infection with frequent skin involvement. Epstein-Barr virus (EBV) and human herpes virus (HHV)-6 are reported to be associated with AITL, but there are conflicting results. OBJECTIVE: We evaluated the association of EBV and HHV-6 with AITL. METHODS: We reviewed the clinical, histological and immunophenotypical features of 19 cases of AITL. Among them, 11 lymph node biopsies of AITL were examined for HHV-6, -7, and -8 by polymerase chain reaction (PCR) using virus-specific primers. In situ hybridization of EBV early region RNA (EBER) was performed and T cell receptor (TCR) gene rearrangement was also investigated in some cases. RESULTS: Among these 19 cases, maculopapular, plaque or nodular skin lesions accompanied AITL in 12 cases. Clonal TCR gene rearrangement was seen in 8/9 cases tested. EBER in situ hybridization was positive in 8 cases (57.1%). Among 7 cases with skin biopsies, five cases were consistent with cutaneous involvement of AITL, 1 case was a drug eruption, and the other case was Kaposi's sarcoma. Except a HHV-8 (+) case who also had Kaposi's sarcoma, all of these cases were negative for HHV-6, -7 and -8. CONCLUSION: Skin manifestation seems to be a cardinal component of AITL, be it in the context of presentation, progression or recurrent disease. Recognition of clinicopathological features of skin lesions in AITL as diagnostic clues should be stressed among dermatologists. The lack of HHV-6, -7 and -8 in lymph node biopsy of AITL argues against a pathogenic role for HHVs in AITL.
Biopsy ; Drug Eruptions ; Gene Rearrangement ; Genes, T-Cell Receptor ; Herpesvirus 4, Human ; Herpesvirus 6, Human ; Herpesvirus 8, Human ; Humans ; In Situ Hybridization ; Lymph Nodes ; Lymphoma, T-Cell ; Lymphoproliferative Disorders ; Polymerase Chain Reaction ; Receptors, Antigen, T-Cell ; RNA ; Sarcoma, Kaposi ; Skin ; Skin Manifestations ; Viruses

No abstract available.
Herpesvirus 4, Human*