A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature

Primary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin’s lymphomas. First described in 1996 in HIV inf...

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Main Authors: Farhan Mohammad, Muhammad Neaman Siddique, Faraz Siddiqui, M. Popalzai, Masoud Asgari, Marcel Odaimi
Format: Article
Language:English
Published: Wiley 2014-01-01
Series:Case Reports in Oncological Medicine
Online Access:http://dx.doi.org/10.1155/2014/436821
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author Farhan Mohammad
Muhammad Neaman Siddique
Faraz Siddiqui
M. Popalzai
Masoud Asgari
Marcel Odaimi
author_facet Farhan Mohammad
Muhammad Neaman Siddique
Faraz Siddiqui
M. Popalzai
Masoud Asgari
Marcel Odaimi
author_sort Farhan Mohammad
collection DOAJ
description Primary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin’s lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi’s sarcoma-associated herpesvirus (KSHV) or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as “HHV-8-unrelated PEL-like lymphoma”. Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.
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issn 2090-6706
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spelling doaj-art-45371759e0e14c30944db1cbdf5362cb2025-02-03T05:45:06ZengWileyCase Reports in Oncological Medicine2090-67062090-67142014-01-01201410.1155/2014/436821436821A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the LiteratureFarhan Mohammad0Muhammad Neaman Siddique1Faraz Siddiqui2M. Popalzai3Masoud Asgari4Marcel Odaimi5Department of Internal Medicine, Staten Island University Hospital, Staten Island, NY 10305, USADepartment of Hematology-Oncology, Staten Island University Hospital, Staten Island, NY 10305, USADepartment of Internal Medicine, Staten Island University Hospital, Staten Island, NY 10305, USADepartment of Hematology-Oncology, Staten Island University Hospital, Staten Island, NY 10305, USADepartment of Pathology and Laboratory Medicine, Staten Island University Hospital, Staten Island, NY 10305, USADepartment of Hematology-Oncology, Staten Island University Hospital, Staten Island, NY 10305, USAPrimary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin’s lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi’s sarcoma-associated herpesvirus (KSHV) or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as “HHV-8-unrelated PEL-like lymphoma”. Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.http://dx.doi.org/10.1155/2014/436821
spellingShingle Farhan Mohammad
Muhammad Neaman Siddique
Faraz Siddiqui
M. Popalzai
Masoud Asgari
Marcel Odaimi
A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature
Case Reports in Oncological Medicine
title A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature
title_full A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature
title_fullStr A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature
title_full_unstemmed A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature
title_short A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature
title_sort unique case of malignant pleuropericardial effusion hhv 8 unrelated pel like lymphoma a case report and review of the literature
url http://dx.doi.org/10.1155/2014/436821
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