Synchronous follicular lymphoma, kaposi sarcoma, and castleman's disease in a HIV-negative patient with EBV and HHV-8 coinfection.
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 cytokine-driven hyperplasia, culminating in lymphoma.
Koreishi
Aashiyana F
AF
Department of Pathology and Radiology, Memorial Sloan-Kettering Cancer Center, New York, USA.
Saenz
Adam J
AJ
Arcila
Maria E
ME
Hedvat
Cyrus
C
Fleming
Stephen
S
Teruya-Feldstein
Julie
J
D000368:Aged; D005871:Castleman Disease / Q000150:complications / Q000473:pathology* / Q000821:virology; D060085:Coinfection / Q000473:pathology*; D003924:Diabetes Mellitus, Type 2 / Q000150:complications; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D005434:Flow Cytometry; D005756:Gastritis / Q000150:complications; D019288:Herpesvirus 8, Human; D006801:Humans; D008224:Lymphoma, Follicular / Q000473:pathology / Q000821:virology*; D009378:Neoplasms, Multiple Primary / Q000473:pathology / Q000821:virology*; D010195:Pancreatitis / Q000150:complications; D016133:Polymerase Chain Reaction; D012514:Sarcoma, Kaposi / Q000473:pathology / Q000821:virology*