CD4-CD8- T-cell polymyositis in a patient with chronic active Epstein-Barr virus infection

Am J Hematol. 2002 Nov;71(3):211-5. doi: 10.1002/ajh.10207.

Abstract

We describe a 17-year-old woman with chronic active Epstein-Barr virus infection (CAEBV), who developed EBV+CD4-CD8- T-cell polymyositis. At 14 years of age, CAEBV was diagnosed with fever, cytopenia, liver dysfunction, and hepatosplenomegaly. Despite the transient remission of interferon-alpha therapy, migratory lesions emerged in back and extremities. MRI indicated polymyositis. Biopsy specimens revealed intramuscular infiltration of CD3+, CD4-, CD8-, CD56-, and EBV-encoded RNA 1+ cells. Circulating CD4-CD8-Vdelta2/Vgamma9 cells increased. gammadeltaT-cells contained 20-200 times higher EBV-DNA (2 x 10(4) copies/microgDNA) than alphabetaT-cells or NK-cells. The ominous polymyositis might denote the musculotropic invasion of EBV+gammadeltaT-cell lymphoproliferative disease as a consequence of CAEBV.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • CD4-Positive T-Lymphocytes / pathology*
  • CD8-Positive T-Lymphocytes / pathology*
  • Chronic Disease
  • Epstein-Barr Virus Infections*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Polymyositis / blood*
  • Polymyositis / diagnosis
  • Polymyositis / pathology
  • Polymyositis / virology*