Severe degenerative change of multiple organs mediated by chronic active Epstein-Barr virus infection with infected T-cell expansion

Michihide Tokuhira, Atsushi Iizuka, Reiko Watanabe, Naoya Sekiguchi, Norihide Sato, Chen Kang Chien, Yasunobu Sekiguchi, Tomoe Nemoto, Kyoko Hanzawa, Jun Ichi Tamaru, Shinji Itoyama, Hiroshi Suzuki, Tsutomu Takeuchi, Shigehisa Mori, Masahiro Kizaki

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4 Citations (Scopus)

Abstract

We here report the case of a young Japanese woman diagnosed with chronic active Epstein-Barr virus (EBV) infection. Intensive therapy with the CHOP regimen was partially able to control virus expansion, but various central nervous system symptoms appeared and gradually progressed. EBV-encoded RNA, detected using in situ hybridization, disclosed the presence of EBV in liver and bone marrow tissue, and real-time PCR revealed the presence of EBV in the cerebrospinal fluid (CSF) and serum. CD3+CD4+CD8-CD56- T-cell expansion in the peripheral blood (PB) and CSF was also observed. Atrophic brain changes were progressive, and the patient died of central nervous system disturbance and pulmonary hemorrhage a year after diagnosis. Autopsy revealed that EBV-infected T lymphocytes with a phenotype similar to those seen in PB and CSF had infiltrated multiple organs: the lymph nodes, bone marrow, endocardium, pericardium, myocardium, spleen, liver, and spinal cord. There have been few previous reports of severe degenerative changes in the myocardium, liver, and spinal cord in patients with EBV infection. Although EBV occasionally infiltrates the central nervous system and brain, atrophic changes mediated by EBV are rare. The autopsy results of this case suggest the possibility of EBV-mediated, severe degenerative changes in multiple organs.

Original languageEnglish
Pages (from-to)520-526
Number of pages7
JournalInternational journal of hematology
Volume87
Issue number5
DOIs
Publication statusPublished - 2008 Jun 1
Externally publishedYes

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Keywords

  • Chronic active Epstein-Barr virus infection
  • Degenerative change
  • Encephalomyelitis
  • Hepatitis
  • Lymphoproliferative disorder

ASJC Scopus subject areas

  • Hematology

Cite this

Tokuhira, M., Iizuka, A., Watanabe, R., Sekiguchi, N., Sato, N., Chien, C. K., Sekiguchi, Y., Nemoto, T., Hanzawa, K., Tamaru, J. I., Itoyama, S., Suzuki, H., Takeuchi, T., Mori, S., & Kizaki, M. (2008). Severe degenerative change of multiple organs mediated by chronic active Epstein-Barr virus infection with infected T-cell expansion. International journal of hematology, 87(5), 520-526. https://doi.org/10.1007/s12185-008-0069-4