Clinicopathologic Characteristics of HHV8 Negative Effusion Based Lymphomas, a Distinct Entity: Report of 4 Cases and Review of the Literature
Serge Alexanian, Sheeja T Pullarkat. David Geffen School of Medicine, University of California, Los Angeles, CA
Background: Effusion based lymphomas that are Human Herpes Virus-8 (HHV8) positive have been well described as Primary Effusion Lymphoma (PEL). Cases of HHV8 negative effusion based lymphomas (HHV8 negative EBL) morphologically resembling PEL have been reported in the literature and in many cases have been (mis)classified as PEL-like lymphomas. Herein we describe a series of four patients as well as the largest literature review to date of HHV8 negative EBL and attempt to review their clinicopathologic characteristics.
Design: Cases indexed on Pubmed were collected utilizing the following criteria: lymphomas arising entirely within serous cavities; lack of associated solid lymphoid malignancies; cytomorphologic features resembling classic PEL; lack of HHV8 expression. Cases with either Burkittoid morphology or t(8:22)(q24;q11) were deemed effusion variants of Burkitt Lymphoma and excluded. Four new/unreported cases from our institution were added for a total of 44.
Results: The clinical and immunophenotypic characteristics of HHV8 negative EBL are compared against traditional PEL in Figure 1:
Conclusions: Our analysis demonstrates that HHV8 negative EBL, while sharing cytomorphologic characteristics with PEL, is a distinct entity: lymphoma cells are HHV8 negative and express pan-B cell antigens; patients are older with less male predominance, are generally HIV negative, and often have an underlying medical condition leading to fluid overload; clinical outcomes and response to therapy are much improved.
Furthermore, the striking association with underlying fluid overload states predating the malignant effusion raises the possibility that these lymphomas are in fact secondary to chronic serosal stimulation, much like pyothorax association lymphomas. Given the distinct clinicopathologic features of HHV8 negative EBL, a provisional heading under the aegis of Diffuse Large B-Cell Lymphoma may be a consideration.
Tuesday, March 20, 2012 1:00 PM
Poster Session IV # 205, Tuesday Afternoon