KSHV/HHV8-negative effusion-based lymphoma, a distinct entity associated with fluid overload states

Am J Surg Pathol. 2013 Feb;37(2):241-9. doi: 10.1097/PAS.0b013e318267fabc.

Abstract

Human herpesvirus-8 (HHV8)-positive effusion-based lymphomas have been termed primary effusion lymphoma (PEL) in the WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. Kaposi sarcoma herpesvirus (KSHV)/HHV8-negative effusion-based lymphomas (KSHV/HHV8-negative EBLs) resembling PELs have been reported in the literature and in many cases have been (mis)classified as PEL-like lymphomas. Herein, we present a series of cases and a review of KSHV/HHV8-negative EBLs. This lymphoma, although cytomorphologically resembling PEL, is a distinct entity with characteristic clinical and pathologic features. Patients are older, generally human immunodeficiency virus negative and not immunosuppressed, frequently hepatitis C positive compared with the population baseline, and often have an underlying medical condition leading to fluid overload. The lymphoma cells express pan-B-cell antigens in 86.7%, and CD20 is expressed in 71.1% of the cases. The lymphoma is often of germinal center B or mixed germinal center B/activated B-cell signature with the Hans classifier, and Epstein-Barr virus is positive in nearly 30% of cases. Rare T-cell lymphomas were also reported. Clinical outcomes and response to therapy, including isolated aspiration, are relatively favorable compared with cases of PEL. We suggest that HHV8-negative effusion-based lymphoma is a distinct entity associated with fluid overload states.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antigens, CD20 / metabolism
  • Biomarkers, Tumor / metabolism
  • Fatal Outcome
  • Female
  • Herpesvirus 8, Human / isolation & purification*
  • Humans
  • Lymphoma, Primary Effusion / complications
  • Lymphoma, Primary Effusion / metabolism
  • Lymphoma, Primary Effusion / pathology*
  • Lymphoma, Primary Effusion / therapy
  • Male
  • Middle Aged
  • Retrospective Studies
  • Sarcoma, Kaposi / complications
  • Sarcoma, Kaposi / metabolism
  • Sarcoma, Kaposi / pathology*
  • Treatment Outcome
  • Water-Electrolyte Imbalance / complications
  • Water-Electrolyte Imbalance / metabolism
  • Water-Electrolyte Imbalance / pathology*

Substances

  • Antigens, CD20
  • Biomarkers, Tumor