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

Research output: Contribution to journalArticle

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
Externally publishedYes

Fingerprint

Epstein-Barr Virus Infections
Human Herpesvirus 4
T-Lymphocytes
Cerebrospinal Fluid
Central Nervous System
Autopsy
Liver
Spinal Cord
Myocardium
Bone Marrow
Endocardium
Pericardium
Brain
In Situ Hybridization
Real-Time Polymerase Chain Reaction
Spleen
Lymph Nodes
RNA
Hemorrhage
Viruses

Keywords

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

ASJC Scopus subject areas

  • Hematology

Cite this

Severe degenerative change of multiple organs mediated by chronic active Epstein-Barr virus infection with infected T-cell expansion. / Tokuhira, Michihide; Iizuka, Atsushi; Watanabe, Reiko; Sekiguchi, Naoya; Sato, Norihide; Chien, Chen Kang; Sekiguchi, Yasunobu; Nemoto, Tomoe; Hanzawa, Kyoko; Tamaru, Jun Ichi; Itoyama, Shinji; Suzuki, Hiroshi; Takeuchi, Tsutomu; Mori, Shigehisa; Kizaki, Masahiro.

In: International Journal of Hematology, Vol. 87, No. 5, 06.2008, p. 520-526.

Research output: Contribution to journalArticle

Tokuhira, M, Iizuka, A, Watanabe, R, Sekiguchi, N, Sato, N, Chien, CK, Sekiguchi, Y, Nemoto, T, Hanzawa, K, Tamaru, JI, 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, vol. 87, no. 5, pp. 520-526. https://doi.org/10.1007/s12185-008-0069-4
Tokuhira, Michihide ; Iizuka, Atsushi ; Watanabe, Reiko ; Sekiguchi, Naoya ; Sato, Norihide ; Chien, Chen Kang ; Sekiguchi, Yasunobu ; Nemoto, Tomoe ; Hanzawa, Kyoko ; Tamaru, Jun Ichi ; Itoyama, Shinji ; Suzuki, Hiroshi ; Takeuchi, Tsutomu ; Mori, Shigehisa ; Kizaki, Masahiro. / Severe degenerative change of multiple organs mediated by chronic active Epstein-Barr virus infection with infected T-cell expansion. In: International Journal of Hematology. 2008 ; Vol. 87, No. 5. pp. 520-526.
@article{82e141baf7704ae988c1bb6b7a336e77,
title = "Severe degenerative change of multiple organs mediated by chronic active Epstein-Barr virus infection with infected T-cell expansion",
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.",
keywords = "Chronic active Epstein-Barr virus infection, Degenerative change, Encephalomyelitis, Hepatitis, Lymphoproliferative disorder",
author = "Michihide Tokuhira and Atsushi Iizuka and Reiko Watanabe and Naoya Sekiguchi and Norihide Sato and Chien, {Chen Kang} and Yasunobu Sekiguchi and Tomoe Nemoto and Kyoko Hanzawa and Tamaru, {Jun Ichi} and Shinji Itoyama and Hiroshi Suzuki and Tsutomu Takeuchi and Shigehisa Mori and Masahiro Kizaki",
year = "2008",
month = "6",
doi = "10.1007/s12185-008-0069-4",
language = "English",
volume = "87",
pages = "520--526",
journal = "International Journal of Hematology",
issn = "0925-5710",
publisher = "Springer Japan",
number = "5",

}

TY - JOUR

T1 - Severe degenerative change of multiple organs mediated by chronic active Epstein-Barr virus infection with infected T-cell expansion

AU - Tokuhira, Michihide

AU - Iizuka, Atsushi

AU - Watanabe, Reiko

AU - Sekiguchi, Naoya

AU - Sato, Norihide

AU - Chien, Chen Kang

AU - Sekiguchi, Yasunobu

AU - Nemoto, Tomoe

AU - Hanzawa, Kyoko

AU - Tamaru, Jun Ichi

AU - Itoyama, Shinji

AU - Suzuki, Hiroshi

AU - Takeuchi, Tsutomu

AU - Mori, Shigehisa

AU - Kizaki, Masahiro

PY - 2008/6

Y1 - 2008/6

N2 - 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.

AB - 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.

KW - Chronic active Epstein-Barr virus infection

KW - Degenerative change

KW - Encephalomyelitis

KW - Hepatitis

KW - Lymphoproliferative disorder

UR - http://www.scopus.com/inward/record.url?scp=46149109254&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=46149109254&partnerID=8YFLogxK

U2 - 10.1007/s12185-008-0069-4

DO - 10.1007/s12185-008-0069-4

M3 - Article

VL - 87

SP - 520

EP - 526

JO - International Journal of Hematology

JF - International Journal of Hematology

SN - 0925-5710

IS - 5

ER -