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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2014-01-01
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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. |
format | Article |
id | doaj-art-45371759e0e14c30944db1cbdf5362cb |
institution | Kabale University |
issn | 2090-6706 2090-6714 |
language | English |
publishDate | 2014-01-01 |
publisher | Wiley |
record_format | Article |
series | Case Reports in Oncological Medicine |
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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