Clinicopathological characteristics compared between primary effusion lymphoma and human herpesvirus 8 (HHV8)-unrelated primary effusion lymphoma-like lymphoma.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e19024-e19024
Author(s):  
Junghoon Shin ◽  
Youngil Koh
Keyword(s):  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
B Cell Lymphoma ◽  
Clinicopathological Characteristics ◽  
Small Sample ◽  
Human Herpesvirus 8 ◽  
Vitreous Fluid ◽  
Female Ratio ◽  
Herpesvirus 8 ◽  
Significant Difference

e19024 Background: Primary effusion lymphoma (PEL) and human herpesvirus 8 (HHV8)-unrelated primary effusion lymphoma-like lymphoma (PEL-LL) are among the most rare subtypes of non-Hodgkin B-cell lymphoma. Although a few number of case reports and small case series have suggested a better outcome of PEL-LL compared to PEL, systematic comparison of prognosis between PEL and PEL-LL has never been conducted owing to their rare incidence. Methods: We consecutively collected PEL and PEL-LL patients diagnosed between 2000 and 2016 in 10 institutions in South Korea. All patients were cytogically confirmed and received systemic evaluation to exclude detectable tumor formation. Clinicopathological characteristics and prognosis were compared. Results: A total of 12 PEL and 4 PEL-LL cases were analyzed. Median age at diagnosis was 68.5 (range, 40-88) and 68.5 (range, 59-77) for PEL and PEL-LL, respectively. Male-to-female ratio was 7:5 and 3:1 in each group. Pleural effusion was the most common presentation in both groups. One patient in PEL-LL group presented with vitreous fluid containing lymphoma cells. Seven and two PEL patients were treated with CHOP and CVP-based chemotherapy, respectively and 3 did not receive chemotherapy due to poor performance. Three PEL-LL patients were treated with R-CHOP and the remaining 1 patient with malignant vitreous fluid received high dose methotrexate. One PEL patient received autologous hematopoietic stem cell transplantation after second remission and lived over 14 years thereafter. With estimated median follow-up duration of 99.6 and 20.7 months for PEL and PEL-LL respectively, 3 and 1 death occurred during the entire period in each group. Median overall survival was 119.6 and 66.5 months in PEL and PEL-LL group respectively without statistically significant difference. Conclusions: In our study, long-term prognosis of PEL and PEL-LL showed no difference. This result might be attributed to 2 remarkably long-lived patients in PEL group (longer than 174 and 123 months, respectively) and low statistical power owing to the small sample size.

scholarly journals Spectrum of Kaposi's Sarcoma-Associated Herpesvirus, or Human Herpesvirus 8, Diseases

2002 ◽  
Vol 15 (3) ◽  
pp. 439-464 ◽  
Author(s):  
Dharam V. Ablashi ◽  
Louise G. Chatlynne ◽  
James E. Whitman, ◽  
Ethel Cesarman
Keyword(s):  
Dna Sequences ◽  
Kaposi's Sarcoma ◽  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
Kaposi’S Sarcoma ◽  
B Cell Lymphoma ◽  
Human Herpesvirus 8 ◽  
Herpesvirus 8 ◽  
Kaposi's Sarcoma Associated Herpesvirus ◽  
Kaposi’S Sarcoma Associated Herpesvirus

SUMMARY Human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV), discovered in 1994, is a human rhadinovirus (gamma-2 herpesvirus). Unlike other human herpesviruses (herpes simplex virus, Epstein-Barr virus, varicella-zoster virus, cytomegalovirus, HHV-6, and HHV-7), it is not widespread in the general population and has many unique proteins. HHV-8 is strongly associated with all subtypes of Kaposi's sarcoma (KS), multicentric Castleman's disease, and a rare form of B-cell lymphoma, primary effusion lymphoma. In addition, HHV-8 DNA sequences have been found in association with other diseases, but the role of the virus in these diseases is largely unconfirmed and remains controversial. The seroprevalence of HHV-8, based on detection of latent and lytic proteins, is 2 to 5% in healthy donors except in certain geographic areas where the virus is endemic, 80 to 95% in classic KS patients, and 40 to 50% in HIV-1 patients without KS. This virus can be transmitted both sexually and through body fluids (e.g., saliva and blood). HHV-8 is a transforming virus, as evidenced by its presence in human malignancies, by the in vitro transforming properties of several of its viral genes, and by its ability to transform some primary cells in culture. It is not, however, sufficient for transformation, and other cofactors such as immunosuppressive cytokines are involved in the development of HHV-8-associated malignancies. In this article, we review the biology, molecular virology, epidemiology, transmission, detection methods, pathogenesis, and antiviral therapy of this newly discovered human herpesvirus.

scholarly journals HUMAN HERPESVIRUS 8 AND LYMPHOPROLIFERATIVE DISEASES

2018 ◽  
Vol 10 ◽  
pp. e2018061 ◽  
Author(s):  
Maria Luisa Calabrò ◽  
Ronit Sarid
Keyword(s):  
Developmental Stages ◽  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
B Cell Lymphoma ◽  
Intermediate Stage ◽  
Lymphoproliferative Disorders ◽  
Human Herpesvirus 8 ◽  
Herpesvirus 8 ◽  
Almost All ◽  
Hiv 1

The spectrum of lymphoproliferative disorders linked to human herpesvirus 8 (HHV-8) infection has been constantly increasing since the discovery of its first etiologic association with primary effusion lymphoma (PEL). PEL is a rapidly progressing non-Hodgkin’s B-cell lymphoma that develops in body cavities in an effusional form. With the increase in the overall survival of PEL patients, as well as the introduction of HHV-8 surveillance in immunocompromised patients, the extracavitary, solid counterpart of PEL was later identified. Moreover, virtually all plasmablastic variants of multicentric Castleman’s disease (MCD) developing in HIV-1-infected individuals harbor HHV-8, providing a strong etiologic link between MCD and this oncogenic herpesvirus. Two other pathological conditions develop in HIV-1-infected persons concomitantly with MCD: MCD with plasmablastic clusters and MCD-associated plasmablastic lymphoma, the first likely representing an intermediate stage preceding the full neoplastic stage. MCD in leukemic phase has also been described, albeit much less commonly. The germinotropic lymphoproliferative disorder (GLPD) may resemble extracavitary PEL, but develops in immune competent HHV8-infected individuals, and, unlike the disorders described above, it responds well to conventional therapies. Almost all HHV-8-mediated lymphoproliferative disorders are the result of an interaction between HHV-8 infection and a dysregulated immunological system, leading to the formation of inflammatory niches in which B cells, at different developmental stages, are infected, proliferate and may eventually shift from a polyclonal state to a monoclonal/neoplastic disorder. Herein, we describe the association between HHV-8 and lymphoproliferative disorders and highlight the predominant distinctive features of each disease.

scholarly journals The targeting of primary effusion lymphoma cells for apoptosis by inducing lytic replication of human herpesvirus 8 while blocking virus production

Blood ◽  
2005 ◽  
Vol 105 (10) ◽  
pp. 4028-4034 ◽  
Author(s):  
Carmen M. Klass ◽  
Laurie T. Krug ◽  
Veronika P. Pozharskaya ◽  
Margaret K. Offermann
Keyword(s):  
Tumor Cells ◽  
Infectious Virus ◽  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
Virus Production ◽  
B Cell Lymphoma ◽  
Lytic Replication ◽  
Human Herpesvirus 8 ◽  
Viral Transcript ◽  
Herpesvirus 8

AbstractPrimary effusion lymphoma (PEL) is a B-cell lymphoma in which human herpesvirus-8 (HHV-8) is found within all tumor cells and represents a target for selectively destroying tumor cells. HHV-8 is latent in most PEL cells and, hence, resistant to antiviral agents that inhibit lytic replication. We demonstrate that PEL cell lines containing HHV-8 without and with coinfection with Epstein-Barr virus responded to the antiseizure medication valproate with entry into the lytic cascade and production of infectious virus. Minimal cell death occurred when noninfected BL-41 cells were incubated with valproate, whereas apoptosis occurred in response to valproate in PELs that supported lytic replication of HHV-8. The anti-viral agents ganciclovir and phosphonoformic acid (PFA) blocked valproate-induced production of infectious virus without blocking entry into the lytic cascade, and apoptosis occurred at levels that were as high as when virus production was not blocked. Ganciclovir and PFA also prevented most valproate-induced expression of the late lytic viral transcript open reading frame 26 (ORF-26), but they did not block the induction of either viral interleukin-6 (vIL-6) or viral G protein-coupled receptor (vGPCR). These studies provide evidence that incubation of PELs with valproate in the presence of ganciclovir or PFA can selectively target tumor cells for apoptosis without increasing viral load.

Association of Primary Pleural Effusion Lymphoma of T-Cell Origin and Human Herpesvirus 8 in a Human Immunodeficiency Virus–Seronegative Man

2001 ◽  
Vol 125 (9) ◽  
pp. 1246-1248 ◽  
Author(s):  
Emmanuèle Lechapt-Zalcman ◽  
Dominique Challine ◽  
Marie-Hélène Delfau-Larue ◽  
Corinne Haioun ◽  
Dominique Desvaux ◽  
...  
Keyword(s):  
Human Immunodeficiency Virus ◽  
T Cell ◽  
Dna Sequences ◽  
Primary Effusion Lymphoma ◽  
Polymerase Chain Reaction Amplification ◽  
Human Herpesvirus ◽  
Body Cavity ◽  
Human Herpesvirus 8 ◽  
Herpesvirus 8 ◽  
Immunodeficiency Virus

Abstract We describe a case of an 87-year-old human immunodeficiency virus (HIV)–negative man who developed a primary pleural lymphoma without any identifiable tumor mass associated with human herpesvirus 8 (HHV-8) infection. A large T-cell lymphoma was diagnosed based on morphologic, immunophenotypic, and molecular findings. The HHV-8 DNA sequences were detected using specific polymerase chain reaction amplification in the lymphomatous effusion. Study of the patient's serum confirmed the HHV-8 infection. This case report displays the characteristic features of HHV-8–related body cavity-based lymphoma/primary effusion lymphoma previously reported in HIV-seronegative patients, except that it is of T-cell origin. Whether this case may be included or not within the primary effusion lymphoma entity, the association of a pleural T-cell non-Hodgkin lymphoma with HHV-8 infection raises the question of the possible occurrence of T cells as the target of malignant transformation associated with HHV-8 infection.

scholarly journals A Systems Biology Approach To Identify the Combination Effects of Human Herpesvirus 8 Genes on NF-κB Activation

2009 ◽  
Vol 83 (6) ◽  
pp. 2563-2574 ◽  
Author(s):  
Andreas Konrad ◽  
Effi Wies ◽  
Mathias Thurau ◽  
Gaby Marquardt ◽  
Elisabeth Naschberger ◽  
...  
Keyword(s):  
Systems Biology ◽  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
Etiologic Agent ◽  
Lytic Replication ◽  
Fine Tuning ◽  
Human Herpesvirus 8 ◽  
Systematic Analysis ◽  
Herpesvirus 8 ◽  
Infected Cells

ABSTRACT Human herpesvirus 8 (HHV-8) is the etiologic agent of Kaposi's sarcoma and primary effusion lymphoma. Activation of the cellular transcription factor nuclear factor-kappa B (NF-κB) is essential for latent persistence of HHV-8, survival of HHV-8-infected cells, and disease progression. We used reverse-transfected cell microarrays (RTCM) as an unbiased systems biology approach to systematically analyze the effects of HHV-8 genes on the NF-κB signaling pathway. All HHV-8 genes individually (n = 86) and, additionally, all K and latent genes in pairwise combinations (n = 231) were investigated. Statistical analyses of more than 14,000 transfections identified ORF75 as a novel and confirmed K13 as a known HHV-8 activator of NF-κB. K13 and ORF75 showed cooperative NF-κB activation. Small interfering RNA-mediated knockdown of ORF75 expression demonstrated that this gene contributes significantly to NF-κB activation in HHV-8-infected cells. Furthermore, our approach confirmed K10.5 as an NF-κB inhibitor and newly identified K1 as an inhibitor of both K13- and ORF75-mediated NF-κB activation. All results obtained with RTCM were confirmed with classical transfection experiments. Our work describes the first successful application of RTCM for the systematic analysis of pathofunctions of genes of an infectious agent. With this approach, ORF75 and K1 were identified as novel HHV-8 regulatory molecules on the NF-κB signal transduction pathway. The genes identified may be involved in fine-tuning of the balance between latency and lytic replication, since this depends critically on the state of NF-κB activity.

Prognostic Factors and Outcome of Human Herpesvirus 8–Associated Primary Effusion Lymphoma in Patients With AIDS

2005 ◽  
Vol 23 (19) ◽  
pp. 4372-4380 ◽  
Author(s):  
Emmanuelle Boulanger ◽  
Laurence Gérard ◽  
Jean Gabarre ◽  
Jean-Michel Molina ◽  
Christophe Rapp ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Survival Rate ◽  
Median Survival ◽  
Primary Effusion Lymphoma ◽  
Performance Status ◽  
Human Herpesvirus ◽  
Disease Free Survival ◽  
Hazard Ratio ◽  
Human Herpesvirus 8 ◽  
Herpesvirus 8

PurposePrimary effusion lymphoma (PEL) is a rare high-grade B-cell non-Hodgkin's lymphoma associated with Kaposi sarcoma–associated herpesvirus/human herpesvirus 8 (KSHV/HHV-8) infection, and is mostly observed in the course of HIV infection. The prognosis is poor, with reported median survival time shorter than 6 months. To date, no prognostic factor has been identified in this subset of lymphoma.Patients and MethodsWe describe here a large series of HIV-infected patients with PEL, including 28 cases diagnosed in six centers during an 11-year time period. Prognosis analysis was performed using a Cox proportional hazard regression model. Statistically significant covariates were further analyzed in a forward, stepwise multivariate model.ResultsAfter a median follow-up of 3.8 years (range, 10 months to 10.8 years), nine patients (32%) were still alive, and eight of them remained progression free. The median survival was 6.2 months, and the 1-year overall survival rate was 39.3%. Fourteen patients (50%) achieved complete remission, with a 1-year disease-free survival rate at 78.6%. In a multivariate analysis, only a performance status more than 2 (hazard ratio, 5.84; 95% CI, 1.76 to 19.33) and the absence of highly active antiretroviral therapy (HAART) before PEL diagnosis (hazard ratio, 3.26; 95% CI, 1.14 to 9.34) were found to be independent predictors for shorter survival.ConclusionBased on a retrospective series of 28 patients, two prognostic factors were identified as being independently associated with impaired clinical outcome in HIV-related PEL—(1) a poor performance status and (2) the absence of HAART before PEL diagnosis.

scholarly journals Human herpesvirus 8‐related primary effusion lymphoma in four HIV‐uninfected patients without organ transplantation

2019 ◽  
Vol 8 (1) ◽  
Author(s):  
Hung‐I Kuo ◽  
Yu‐Ting Yu ◽  
Kung‐Chao Chang ◽  
Po‐Lan Su ◽  
Sin‐Syue Li ◽  
...  
Keyword(s):  
Organ Transplantation ◽  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
Human Herpesvirus 8 ◽  
Herpesvirus 8

scholarly journals Differential Expression of Viral Bcl-2 Encoded by Kaposi's Sarcoma-Associated Herpesvirus and Human Bcl-2 in Primary Effusion Lymphoma Cells and Kaposi's Sarcoma Lesions

2002 ◽  
Vol 76 (5) ◽  
pp. 2551-2556 ◽  
Author(s):  
Isabelle Widmer ◽  
Marion Wernli ◽  
Felix Bachmann ◽  
Fred Gudat ◽  
Gieri Cathomas ◽  
...  
Keyword(s):  
Kaposi's Sarcoma ◽  
Mononuclear Cells ◽  
Primary Effusion Lymphoma ◽  
Human Herpesvirus ◽  
Kaposi’S Sarcoma ◽  
Human Herpesvirus 8 ◽  
Lymphoma Cells ◽  
Herpesvirus 8 ◽  
Primary Effusion Lymphoma Cell ◽  
Spindle Cells

ABSTRACT Expression of human herpesvirus 8 viral Bcl-2 protein was demonstrated in spindle cells of late-stage Kaposi's sarcoma lesions but not in primary effusion lymphoma cell lines. In contrast, strong expression of human Bcl-2 was found in stimulated primary effusion lymphoma cells, whereas in Kaposi's sarcoma lesions preferential mononuclear cells, and to a lesser extent spindle cells, stained positive.

Sign in / Sign up

Export Citation Format

Share Document