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International Journal of Surgical Pathology
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Article

Synchronous Follicular Lymphoma, Kaposi Sarcoma, and Castleman's Disease in a HIV-Negative Patient With EBV and HHV-8 Coinfection

Aashiyana Koreishi, Adam J Saenz, Maria E. Arcila, Cyrus Hedvat, Stephen Fleming, and Julie Teruya-Feldstein, MD*

* To whom correspondence should be addressed. E-mail: feldstej{at}mskcc.org.


  Abstract
The authors describe the case of a 65-year-old woman who was HIV negative and had a lymph node biopsy that showed concurrent follicular lymphoma (FL; grade 3A), Kaposi sarcoma (KS), and Castleman’s disease (CD) with coinfection by human herpes virus-8 (HHV-8) and Epstein-Barr virus (EBV). The lymphoma was positive for CD20, CD10, and BCL6 and negative for BCL2. Flow cytometry showed a clonal lambda B-cell population, and polymerase chain reaction (PCR) showed a clonal immunoglobulin heavy chain gene rearrangement, confirming a neoplastic B-cell process. Focally, the FL component showed numerous EBER1-positive cells, with rare HHV-8-positive cells. The KS component showed strong HHV-8 expression with rare EBER1-positive cells. The CD component showed scattered HHV-8, viral interleukin-6, and EBER1-positive cells. The simultaneous occurrence of a FL, KS, and CD in an HIV-negative patient expands the spectrum of HHV-8-positive neoplasms and suggests the possibility of HHV-8 rendering mature B-cells hyperresponsive to antigenic stimulation, providing an expanded target for second site mutations or cytokinedriven hyperplasia, culminating in lymphoma.

First published on August 5, 2009
International Journal of Surgical Pathology 2009, doi:10.1177/1066896909341803
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