scholarly journals HHV8-Negative Primary Effusion Lymphoma of B-Cell Lineage: Two Cases and a Comprehensive Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-12 ◽  
Author(s):  
Neeraj Saini ◽  
Ephraim P. Hochberg ◽  
Erica A. Linden ◽  
Smita Jha ◽  
Heinz K. Grohs ◽  
...  
Keyword(s):  
B Cell ◽  
English Language ◽  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Cell Lineage ◽  
Kaposi Sarcoma ◽  
Body Cavity ◽  
Lymphoid Cells ◽  
Human Herpes Virus 8 ◽  
Herpès Virus

Primary effusion lymphoma (PEL) is a rare extranodal lymphoma that typically presents in a body cavity in the absence of a detectable tumor mass and that occurs predominantly in immunosuppressed individuals. The neoplastic lymphoid cells are frequently infected with human herpes virus 8 (HHV8), also known as Kaposi sarcoma herpes virus (KSHV). We describe two HIV-negative patients who presented with primary effusion lymphoma of B-cell lineage involving the pleural cavity, but whose tumor cells lacked infection by HHV8. We review the English language literature of HHV8-negative PEL of B-cell lineage and compare these lymphomas to HHV8-associated PEL with regard to clinical and pathological characteristics, therapy, and outcome.

scholarly journals Primary effusion lymphoma occurring in the setting of transplanted patients: a systematic review of a rare, life-threatening post-transplantation occurrence

BMC Cancer ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Magda Zanelli ◽  
Francesca Sanguedolce ◽  
Maurizio Zizzo ◽  
Andrea Palicelli ◽  
Maria Chiara Bassi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Kaposi Sarcoma ◽  
Body Cavity ◽  
Solid Organ ◽  
Human Herpes Virus 8 ◽  
Post Transplant ◽  
Human Herpes Virus ◽  
Herpès Virus

Abstract Background Primary effusion lymphoma is a rare, aggressive large B-cell lymphoma strictly linked to infection by Human Herpes virus 8/Kaposi sarcoma-associated herpes virus. In its classic form, it is characterized by body cavities neoplastic effusions without detectable tumor masses. It often occurs in immunocompromised patients, such as HIV-positive individuals. Primary effusion lymphoma may affect HIV-negative elderly patients from Human Herpes virus 8 endemic regions. So far, rare cases have been reported in transplanted patients. The purpose of our systematic review is to improve our understanding of this type of aggressive lymphoma in the setting of transplantation, focusing on epidemiology, clinical presentation, pathological features, differential diagnosis, treatment and outcome. The role of assessing the viral serological status in donors and recipients is also discussed. Methods We performed a systematic review adhering to the PRISMA guidelines. The literature search was conducted on PubMed/MEDLINE, Web of Science, Scopus, EMBASE and Cochrane Library, using the search terms “primary effusion lymphoma” and “post-transplant”. Results Our search identified 13 cases of post-transplant primary effusion lymphoma, predominantly in solid organ transplant recipients (6 kidney, 3 heart, 2 liver and 1 intestine), with only one case after allogenic bone marrow transplantation. Long-term immunosuppression is important in post-transplant primary effusion lymphoma commonly developing several years after transplantation. Kaposi Sarcoma occurred in association with lymphoma in 4 cases of solid organ recipients. The lymphoma showed the classical presentation with body cavity effusions in absence of tumor masses in 10 cases; 2 cases presented as solid masses, lacking effusions and one case as effusions associated with multiple organ involvement. Primary effusion lymphoma occurring in the setting of transplantation was more often Epstein Barr-virus negative. The prognosis was poor. In addition to chemotherapy, reduction of immunosuppressive treatment, was generally attempted. Conclusions Primary effusion lymphoma is a rare, but often fatal post-transplant complication. Its rarity and the difficulty in achieving the diagnosis may lead to miss this complication. Clinicians should suspect primary effusion lymphoma in transplanted patients, presenting generally with unexplained body cavity effusions, although rare cases with solid masses are described.

scholarly journals Primary Effusion Lymphoma Occurring in the Setting of Transplanted Patients: A Systematic Review of a Rare, Life-Threatening Post-Transplantation Occurrence

2021 ◽  
Author(s):  
Magda Zanelli ◽  
Francesca Sanguedolce ◽  
Maurizio Zizzo ◽  
Andrea Palicelli ◽  
Maria Chiara Bassi ◽  
...  
Keyword(s):  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Kaposi Sarcoma ◽  
Body Cavity ◽  
Solid Organ ◽  
Human Herpes ◽  
Human Herpes Virus 8 ◽  
Post Transplant ◽  
Human Herpes Virus ◽  
Herpès Virus

Abstract Background: Primary effusion lymphoma is a rare, aggressive large B-cell lymphoma strictly linked to infection by Human Herpes virus 8/Kaposi sarcoma-associated herpes virus. In its classic form, it is characterized by body cavities neoplastic effusions without detectable tumor masses. It often occurs in immunocompromised patients, such as HIV-positive individuals. Primary effusion lymphoma may affect HIV-negative elderly patients from Human Herpes virus 8 endemic regions. So far, rare cases have been reported in transplanted patients. The purpose of our systematic review is to improve our understanding of this type of aggressive lymphoma in the setting of transplantation, focusing on epidemiology, clinical presentation, pathological features, differential diagnosis, treatment and outcome. The role of assessing the viral serological status in donors and recipients is also discussed.Methods: Based on the PRISMA guidelines, we carried out a systematic search on PubMed/MEDLINE, Web of Science, Scopus, EMBASE and Cochrane Library using the search terms “primary effusion lymphoma” and “post-transplant”. Results: Our search identified 13 cases of post-transplant primary effusion lymphoma, predominantly in solid organ transplant recipients (6 kidney, 3 heart, 2 liver and 1 intestine), with only one case after allogenic bone marrow transplantation. Long-term immunosuppression is important in post-transplant primary effusion lymphoma commonly developing several years after transplantation. Kaposi Sarcoma occurred in association with lymphoma in 4 cases of solid organ recipients. The lymphoma showed the classical presentation with body cavity effusions in absence of tumor masses in 10 cases; 2 cases presented as solid masses, lacking effusions and one case as effusions associated with multiple organ involvement. Primary effusion lymphoma occurring in the setting of transplantation was more often Epstein Barr-virus negative. The prognosis was poor. In addition to chemotherapy, reduction of immunosuppressive treatment, was generally attempted.Conclusions: Primary effusion lymphoma is a rare, but often fatal post-transplant complication. Its rarity and the difficulty in achieving the diagnosis may lead to miss this complication. Clinicians should suspect primary effusion lymphoma in transplanted patients, presenting generally with unexplained body cavity effusions, although rare cases with solid masses are described.

scholarly journals Biology and management of primary effusion lymphoma

Blood ◽  
2018 ◽  
Vol 132 (18) ◽  
pp. 1879-1888 ◽  
Author(s):  
Kazuyuki Shimada ◽  
Fumihiko Hayakawa ◽  
Hitoshi Kiyoi
Keyword(s):  
Tumor Cells ◽  
Herpes Virus ◽  
Immune Escape ◽  
Primary Effusion Lymphoma ◽  
Kaposi Sarcoma ◽  
Underlying Mechanism ◽  
Immune Defense ◽  
Human Herpes Virus 8 ◽  
Herpès Virus ◽  
Anti Cd20

Abstract Primary effusion lymphoma (PEL) is a rare B-cell malignancy that most often occurs in immunocompromised patients, such as HIV-infected individuals and patients receiving organ transplantation. The main characteristic of PEL is neoplastic effusions in body cavities without detectable tumor masses. The onset of the disease is associated with latent infection of human herpes virus 8/Kaposi sarcoma–associated herpes virus, and the normal counterpart of tumor cells is B cells with plasmablastic differentiation. A condition of immunodeficiency and a usual absence of CD20 expression lead to the expectation of the lack of efficacy of anti-CD20 monoclonal antibody; clinical outcomes of the disease remain extremely poor, with an overall survival at 1 year of ∼30%. Although recent progress in antiretroviral therapy has improved outcomes of HIV-infected patients, its benefit is still limited in patients with PEL. Furthermore, the usual high expression of programmed death ligand 1 in tumor cells, one of the most important immune-checkpoint molecules, results in the immune escape of tumor cells from the host immune defense, which could be the underlying mechanism of poor treatment efficacy. Molecular-targeted therapies for the activating pathways in PEL, including NF-κB, JAK/STAT, and phosphatidylinositol 3-kinase/AKT, have emerged to treat this intractable disease. A combination of immunological recovery from immune deficiency, overcoming the immune escape, and the development of more effective drugs will be vital for improving the outcomes of PEL patients in the future.

Human Herpes Virus 8-Negative Primary Effusion Lymphoma in a Patient With a Ventriculoperitoneal Shunt Tube

2001 ◽  
Vol 74 (3) ◽  
pp. 327-332 ◽  
Author(s):  
Eishi Ashihara ◽  
Chihiro Shimazaki ◽  
Hideyo Hirai ◽  
Tohru Inaba ◽  
Goji Hasegawa ◽  
...  
Keyword(s):  
Ventriculoperitoneal Shunt ◽  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Human Herpes ◽  
Human Herpes Virus 8 ◽  
Shunt Tube ◽  
Human Herpes Virus ◽  
Herpès Virus

A case of primary effusion lymphoma without human herpes virus 8 diagnosed via pericardial fluid

2011 ◽  
Vol 50 (4) ◽  
pp. 220-225
Author(s):  
Hiroshi NOGUCHI ◽  
Fumio HANAMURE ◽  
Tomoko SUEKAWA ◽  
Shinya SATO ◽  
Yuichiro SATO ◽  
...  
Keyword(s):  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Pericardial Fluid ◽  
Human Herpes ◽  
Human Herpes Virus 8 ◽  
Human Herpes Virus ◽  
Herpès Virus

Early Results of a Clinical Trial Using Targeted Oncolytic Virotherapy in Kaposi Sarcoma Herpes Virus (KSHV) Associated Multicentric Castleman’s Disease (MCD).

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 514-514
Author(s):  
Deirdre O’Mahony ◽  
Wendy Bernstein ◽  
Kathy Wyvill ◽  
Karen Aleman ◽  
Denise Whitby ◽  
...  
Keyword(s):  
Median Duration ◽  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Oncolytic Virotherapy ◽  
High Dose ◽  
Hematologic Toxicity ◽  
C Reactive Protein ◽  
Human Herpes Virus 8 ◽  
Reactive Protein ◽  
Herpès Virus

Abstract Human herpes virus 8, also termed KSHV, is an oncogenic gamma herpesvirus associated with Kaposi’s sarcoma (KS), primary effusion lymphoma and MCD. KSHV-associated MCD (KSHV-MCD), a rare B-cell lymphoproliferative disease almost universally found in association with HIV infection, is characterized by recurrent flares of a systemic syndrome of fatigue, fevers, cytopenias, elevated serum C-reactive protein (CRP), and lytic KSHV replication. Prognosis is poor (median survival 14 months). KSHV open reading frames (ORF) 21 and 36 respectively have the ability to phosphorylate zidovudine and ganciclovir, leading to cell death (Cancer Res2007;67(14):7003–10), and this may be exploitable for oncolytic virotherapy. Ten patients (pts) with symptomatic and biopsy confirmed KSHV-associated MCD have been treated with high dose oral zidovudine (HDAZT) 600mg every 6 hours and valganciclovir (VGCV) 900mg every 12 hours. Treatment length of first cycle is dependent on response, and ranges from 7–21 days. Subsequent cycle length is 21 days with 7 treatment days. Pt characteristics: median age 40 (range 33–56); ECOG PS 2 (1–3); median enrollment CD4 count 189 (range 19–1319 UL); median HIV viral load <50 copies/ml3 plasma (range <50 to 27,500). All pts were on highly active antiretroviral therapy; this was adjusted during time pts were on AZT. 8 pts had a history of KS. Median duration of MCD 3.5 months (range 0.5–45 months); seven pts had received at least one prior therapy for MCD (range 1–6). All had MCD-related constitutional symptoms and CRP above 0.8mg/dl (median 13.1, range 1.06–38.7 mg/dl) at treatment initiation. A total of 83 cycles have been administered to date, with a median of 6 (range 4–23) cycles per pt. 9/10 had documented improvement in constitutional symptoms, C-reactive protein levels or cytopenias. Therapy continues in 5 patients who have had sustained responses (median duration on therapy 7 months, range 3–18). Response was only short-lived in 4 (median duration on therapy 4.5 months, range 3.5–6.5) who then received alternative treatments. Grade (gr) 3 or 4 hematologic toxicity not attributable to disease was seen in only 2 patients. Two infectious events occurred, a staphylococcal skin abscess and streptococcal meningitis. There were no neutropenia associated infections. Treatment toxicity included two patients with fatigue (gr3), 1 with nausea (gr3), 1 with transaminitis (gr3) and one with insomnia (gr3). In summary, this preliminary data suggests that HDAZT and VGCV has activity useful in the management of KSHV-associated MCD. Accrual continues.

scholarly journals A case of primary effusion lymphoma associated with human herpes virus 8 in an elderly HIV-seronegative male

2005 ◽  
Vol 44 (4) ◽  
pp. 240-244 ◽  
Author(s):  
Ken-ichirou YONEDA ◽  
Shinobu UMEMURA ◽  
Hitoshi ITOH ◽  
Akihiko SERIZAWA ◽  
Naruaki MATSUI ◽  
...  
Keyword(s):  
Herpes Virus ◽  
Primary Effusion Lymphoma ◽  
Human Herpes ◽  
Human Herpes Virus 8 ◽  
Human Herpes Virus ◽  
Herpès Virus
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