The Hammersmith hospital hematopathology case of the month: hemophagocytic lymphohistiocytosis secondary to Epstein-Barr virus associated T-cell lymphoma

Leuk Lymphoma. 2011 Jun;52(6):1127-32. doi: 10.3109/10428194.2011.563888. Epub 2011 Mar 21.

Abstract

We discuss the clinical presentation, investigations carried out, and the management issues in an elderly patient with hemophagocytic lymphohistiocytosis (HLH). The HLH was associated with an underlying Epstein-Barr virus positive peripheral T-cell lymphoma, not otherwise specified, and the lymphomatous cells harbored translocation t(5;9)(q11.2:p24). The 9p24 region contains the JAK2 gene. The disease had a fulminant course.

Publication types

  • Case Reports
  • Clinical Conference

MeSH terms

  • Aged
  • Antibodies, Viral / immunology
  • Chromosomes, Human, Pair 5 / genetics
  • Chromosomes, Human, Pair 9 / genetics
  • Diagnosis, Differential
  • Epstein-Barr Virus Infections / complications
  • Epstein-Barr Virus Infections / diagnosis*
  • Epstein-Barr Virus Infections / virology
  • Fatal Outcome
  • Herpesvirus 4, Human / genetics
  • Herpesvirus 4, Human / immunology
  • Hospitals
  • Humans
  • In Situ Hybridization
  • Janus Kinase 2 / genetics
  • London
  • Lymphohistiocytosis, Hemophagocytic / diagnosis*
  • Lymphohistiocytosis, Hemophagocytic / etiology
  • Lymphoma, T-Cell, Peripheral / complications
  • Lymphoma, T-Cell, Peripheral / diagnosis*
  • Lymphoma, T-Cell, Peripheral / genetics
  • Male
  • RNA, Viral / genetics
  • Translocation, Genetic

Substances

  • Antibodies, Viral
  • Epstein-Barr virus encoded RNA 1
  • RNA, Viral
  • JAK2 protein, human
  • Janus Kinase 2