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Disorders of the Pleura |

Human Herpes Virus 8 - Unrelated Primary Effusion Lymphoma-Like Lymphoma: Classification, Diagnosis, and Management

Jatan Shah, BS; Natasha Bhimani, MD; Rachel Must-Ettinger, MD; He Zhang, MD
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Ross University School of Medicine, Portsmouth, Dominica


Chest. 2014;146(4_MeetingAbstracts):478A. doi:10.1378/chest.1980155
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Abstract

SESSION TITLE: Pleural Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Primary effusion lymphoma (PEL), a rare subtype of Non-Hodgkin Lymphoma, presents as a lymphomatous effusion of B-cells in body cavities without any detectable mass. WHO classifications propose it to be associated with Human Herpes Virus-8 (HHV-8), however, in the literature, 55 cases of HHV-8- unrelated PEL- like lymphoma have been reported. Here, we report another case, localized to the pleural cavity, differentiate it from other effusion lymphomas and comment on the diagnosis and management options.

CASE PRESENTATION: An 84-year-old ♀ presented with a 2-day history of dyspnea without cough or hemoptysis. Physical examination showed dullness to percussion and diminished breath sounds at the right base. CXR and CT scan revealed large right pleural effusion without any masses. Diagnostic thoracocentesis revealed 1600ml of lymphocyte rich, blood-tinged exudate. Cytological examination showed large atypical lymphoid cells with basophilic cytoplasm, consistent with malignant process. On immunohistochemistry, these cells were + for LCA and - for HHV-8. Flow cytometry confirmed it to be monoclonal B-cell population positive for CD19, CD 20, CD 22, CD 38, and CD45. Serological tests for EBV IgG was + but - for HIV, HCV and HHV-8. SPEP and immunofixation showed a monoclonal IgG λ spike in the γ region suggesting a clonal proliferation of B cells. Further workup to rule out extra cavitary causes were performed. CT scan showed no mass or lymphadenopathy in the abdomen or pelvis. No abnormalities on peripheral blood smear or bone marrow biopsy were noted. The diagnosis of HHV 8-unrelated PEL- like lymphoma was made.

DISCUSSION: There are various types of primary NHL that proliferate within the pleural cavity. Among them, PEL has features that are HHV-8 +, EBV +/-, morphologically immunoblastic/anaplastic, B-cell markers - and commonly occur in HIV+ individuals. Burkitt lymphoma's effusion also present without any mass but they are morphologically medium sized cells with B-cell immunophenotype and are CD10+. Pyothorax-associated lymphoma's effusion consists of B-cells but it occurs after a long standing history of pleural pyothorax and has a detectable tumor mass. The case presented here differs from the above effusion lymphomas and resembles HHV8 unrelated PEL-like lymphoma as it has features that are HHV-8 -, EBV +, large cell morphology, B cell markers + and are not related to HIV infection. Chemotherapy (R-CHOP) is the most promising treatment option with a mean survival time of 1 year and the remission rate of 82.1%.

CONCLUSIONS: It is important to recognize HHV-8 unrelated PEL-like lymphoma and differentiate it from other effusion lymphomas because of the difference in pathogenesis, morphologic- immunophenotypic features and clinical behavior. This would help in initiating the appropriate treatment and hence improve the outcome.

Reference #1: Carbone A, Gloghini A. PEL and HHV8-unrelated effusion lymphomas: classification and diagnosis.Cancer. 2008; 114:225-227

DISCLOSURE: The following authors have nothing to disclose: Jatan Shah, Natasha Bhimani, Rachel Must-Ettinger, He Zhang

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