Primary Effusion Lymphoma

A rare neoplasm of large B-cells usually presenting as serious effusions without detectable tumor masses. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. It is associated with HUMAN HERPESVIRUS 8, most often occurring in the setting of immunodeficiency.

Also Known As:

Networked: 496 relevant articles (12 outcomes, 12 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Kaposi Sarcoma (Kaposi's Sarcoma)
2.	Neoplasms (Cancer)
3.	Acquired Immunodeficiency Syndrome (AIDS)
4.	Liver Cirrhosis (Hepatic Cirrhosis)
5.	Lymphoma (Lymphomas)

Experts

1.	Damania, Blossom: 23 articles (01/2015 - 02/2004)
2.	Katano, Harutaka: 15 articles (08/2015 - 12/2003)
3.	Cesarman, Ethel: 14 articles (10/2015 - 07/2002)
4.	Fujimuro, Masahiro: 13 articles (01/2016 - 01/2003)
5.	Dittmer, Dirk P: 13 articles (06/2012 - 11/2005)
6.	Robertson, Erle S: 11 articles (07/2014 - 01/2006)
7.	Schulz, Thomas F: 10 articles (10/2015 - 10/2003)
8.	Okada, Seiji: 10 articles (03/2015 - 09/2009)
9.	Wang, Ling: 10 articles (01/2014 - 04/2004)
10.	Jung, Jae U: 9 articles (10/2014 - 11/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Primary Effusion Lymphoma:

1.	Multi-centric Castleman's DiseaseIBA 08/01/2002 - "The ISH technique with single stranded probe and TSA would be helpful in clinical pathological diagnosis for HHV8 infected diseases, such as KS, primary effusion lymphoma and multicentric Castleman's disease." 11/01/2002 - "The mechanism by which HHV-8 causes KS, primary effusion lymphoma, and multicentric Castleman's disease is not well understood but is thought to involve a number of molecular events, the study of which should further our understanding of viral oncology. " 10/01/2013 - "HHV-8 is involved in the pathophysiological features of multicentric Castleman's disease (MCD) and primary effusion lymphoma (PEL), both rare B-cell lymphoproliferative diseases. " 04/01/2012 - "Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of two B cell lymphoproliferative diseases, namely primary effusion lymphoma (PEL) and multicentric Castleman's disease (MCD). " 03/01/2009 - "KSHV might also contribute to the pathogenesis of primary effusion lymphoma and multicentric Castleman's disease. "
2.	DNA (Deoxyribonucleic Acid)IBA 03/01/2010 - "HHV-8 DNA levels in effusion from patients with primary effusion lymphoma has been reported to be significantly greater than in blood. " 03/01/1997 - "The sensitivity of this assay was determined by a dilution study performed by diluting KSHV DNA from the KS-1 cells (a primary effusion lymphoma cell line which is estimated to have 16 copies of KSHV per cell) in DNA from a K562 myeloid cell line. " 01/01/2015 - "KSHV-DNA load was positive in only one patient, who died from primary effusion lymphoma. " 08/01/2001 - "Primary effusion lymphoma with herpesvirus 8 DNA in patients coinfected with HIV and hepatitis C virus: a report of 2 cases." 11/01/1999 - "HHV-8 DNA is invariably found in all epidemiological forms of KS and primary effusion lymphomas. "
3.	bortezomib (Velcade)FDA Link 01/01/2013 - "In this study we investigated if bortezomib induced endoplasmic reticulum (ER) stress and activated autophagy in Primary Effusion Lymphoma (PEL) cells and how they influenced cell survival. " 01/01/2013 - "JNK and macroautophagy activation by bortezomib has a pro-survival effect in primary effusion lymphoma cells." 05/01/2008 - "Bortezomib (PS-341) in patients with human herpesvirus 8-associated primary effusion lymphoma." 04/01/2005 - "Velcade displays promising activity in primary effusion lymphoma cells." 10/01/2004 - "Antitumor effects of bortezomib (PS-341) on primary effusion lymphomas."
4.	cidofovir (Vistide)FDA Link 05/01/2010 - "Use of cidofovir for the treatment of HIV-negative human herpes virus-8-associated primary effusion lymphoma." 05/01/2010 - "Intracavitary cidofovir for human herpes virus-8-associated primary effusion lymphoma in an HIV-negative patient." 03/01/2005 - "Treatment of herpesvirus associated primary effusion lymphoma with intracavity cidofovir." 12/01/2006 - "A non-chemotherapy treatment of a primary effusion lymphoma: durable remission after intracavitary cidofovir in HIV negative PEL refractory to chemotherapy." 07/15/2011 - "Cidofovir is an antiviral drug active as antitumoral agent a high doses against the Primary Effusion Lymphoma, a herpesvirus HHV8-associated B-cell lymphoma. "
5.	Ribonuclease P (RNase P)IBA 06/15/2004 - "In this study, we demonstrate that the RNase P-based strategy is effective in blocking gene expression and growth of Kaposi's sarcoma (KS)-associated herpesvirus (KSHV), the causative agent of the leading AIDS-associated neoplasms, such as KS and primary-effusion lymphoma. "
6.	rituximab (Mabthera)FDA Link 04/01/2008 - "[Prolonged survival in a patient with human herpesvirus-8-negative primary effusion lymphoma after combination chemotherapy with rituximab]." 01/01/2008 - "HHV-8/KSHV-negative and CD20-positive primary effusion lymphoma successfully treated by pleural drainage followed by chemotherapy containing rituximab." 08/01/2005 - "Primary effusion lymphoma: successful treatment with highly active antiretroviral therapy and rituximab." 12/01/2004 - "Rituximab is effective for human herpesvirus-8-negative primary effusion lymphoma with CD20 phenotype associated hepatitis C virus-related liver cirrhosis." 10/01/2008 - "Herein, we report a case of an elderly HIV-negative man of Mediterranean origin who was diagnosed with primary effusion lymphoma and responded dramatically to 6 cycles of a combination of bortezomib, pegylated liposomal doxorubicin, and rituximab. "
7.	AntibodiesIBA 05/01/2000 - "However, the specificity and sensitivity of these assays are not completely defined due to the lack of a "gold standard." Although IFAs based on primary effusion lymphoma (PEL) cell lines are used widely, the assays can be confounded by nonspecific reactions against cellular components and potential cross-reaction with antibodies against other herpesviruses. " 01/01/2006 - "Colorimetric caspase assay, flow cytometry, and immunoblotting with antibodies against apoptosis-related molecules revealed that GCV has cytotoxic activity toward KSHV-infected primary effusion lymphoma cells, while ACV has weak or little activity. " 05/01/1999 - "Since regression of KS can be achieved by treatment of the patients with alpha interferon (IFN-alpha), we analyzed the effects of IFN-alpha or anti-IFN-alpha antibodies (Ab) on HHV-8 latently infected primary effusion lymphoma-derived cell lines (BCBL-1 and BC-1) and on peripheral blood mononuclear cells (PBMC) from patients with all forms of KS and from at-risk subjects. " 06/15/2010 - "Distinct profiles of antibodies to Kaposi sarcoma-associated herpesvirus antigens in patients with Kaposi sarcoma, multicentric Castleman disease, and primary effusion lymphoma." 04/10/2000 - "To investigate the expression of human herpesvirus 8 (HHV8)-encoded proteins in the cells of primary effusion lymphoma (PEL), Kaposi's sarcoma (KS) and multicentric Castleman's disease (MCD), nine rabbit polyclonal antibodies to K2, ORF26, K8, K8.1, K10, K11, ORF59, ORF65, and ORF73 were developed. "
8.	Ganciclovir (Cytovene)FDA LinkGeneric 01/01/2014 - "Sustained complete remission of primary effusion lymphoma with adjunctive ganciclovir treatment in an HIV-positive patient." 01/01/2006 - "Apoptotic effect of ganciclovir on primary effusion lymphoma cells infected with Kaposi's sarcoma-associated herpesvirus."
9.	sobuzoxaneIBA 04/01/2004 - "Durable remission by sobuzoxane in an HIV-seronegative patient with human herpesvirus 8-negative primary effusion lymphoma." 07/01/2014 - "[Successful treatment with oral low-dose sobuzoxane and etoposide combined with rituximab in an elderly patient with HHV-8-negative primary effusion lymphoma-like lymphoma]."
10.	lenalidomide (CC 5013)FDA Link 01/01/2014 - "Primary effusion lymphoma in an elderly patient effectively treated by lenalidomide: case report and review of literature."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 10/01/2011 - "The Kaposi's sarcoma-associated herpesvirus is the causative agent of primary effusion lymphoma (PEL), for which cytotoxic chemotherapy represents the standard of care. " 07/01/2009 - "Primary effusion lymphoma (PEL) is an incurable, aggressive B-cell malignancy that develops rapid resistance to conventional chemotherapy. " 04/15/2007 - "Primary effusion lymphoma (PEL) is an incurable, aggressive B-cell malignancy that develops rapid resistance to conventional chemotherapy. " 11/10/2008 - "Remission after radiotherapy for a patient with chemotherapy-refractory HIV-associated primary effusion lymphoma." 01/01/2009 - "Primary effusion lymphoma with cardiac involvement in HIV positive patient-complete response and long survival with chemotherapy and HAART."
2.	Heterologous Transplantation (Xenotransplantation) 01/01/2014 - "Systematic analysis of a xenograft mice model for KSHV+ primary effusion lymphoma (PEL)." 07/20/2010 - "Efficacy of bortezomib in a direct xenograft model of primary effusion lymphoma." 10/01/2011 - "Furthermore, in a xenograft-based primary effusion lymphoma (PEL) mouse model, in vivo KSHV reactivation is also H(2)O(2)-dependent and can be suppressed by antioxidants. " 02/01/2010 - "KSHV-associated primary effusion lymphomas (PELs) secrete high levels of vascular endothelial growth factor (VEGF) and viral interleukin-6 (vIL-6) in vitro and VEGF, vIL-6, and basic-fibroblast growth factor (b-FGF) in mouse xenografts. "
3.	Radiotherapy 11/10/2008 - "Remission after radiotherapy for a patient with chemotherapy-refractory HIV-associated primary effusion lymphoma."
4.	Transplants (Transplant) 02/01/2015 - "Primary effusion lymphoma with aberrant T-cell phenotype in an iatrogenically immunosuppressed renal transplant male: cytologic diagnosis in peritoneal fluid." 10/01/2015 - "We report on the case of a 72-year-old human immunodeficiency virus-negative male with a hepatic transplant 10 years prior, who presented with a symptomatic right-sided pleural effusion and was found to have primary effusion lymphoma by flow cytometric and cytopathologic examination. " 06/28/2011 - "Others, like primary effusion lymphoma and polymorphic post-transplant lymphoproliferative disorder are rarely seen outside the context of a specific immunodeficient state. " 12/01/2009 - "KHSV(-) EBV(-) post-transplant effusion lymphoma with plasmablastic features: variant of primary effusion lymphoma?" 10/27/2011 - "Solid organ transplant recipients from HHV-8 endemic regions may develop HHV-8 reactivation or primary infection and manifest with Kaposi's sarcoma or less commonly primary effusion lymphoma and Castleman's disease; these neoplastic diseases are reported much less commonly in low-prevalent areas. "
5.	Transplantation (Transplant Recipients) 04/01/2012 - "A rare case of HHV-8-positive/HIV-negative/EBV-negative primary effusion lymphoma in a renal transplant recipient." 04/01/2010 - "[Primary effusion lymphoma in two kidney transplant recipients]." 04/01/2008 - "Pleural primary effusion lymphoma in a renal transplant recipient." 08/13/1998 - "Primary-effusion lymphoma and Kaposi's sarcoma in a cardiac-transplant recipient." 02/01/2013 - "HHV-8 is an oncogenic gamma-herpesvirus that causes Kaposi's sarcoma, Castleman's disease and primary effusion lymphomas in transplant recipients. "