Severe degenerative change of multiple organs mediated by chronic active Epstein-Barr virus infection with infected T-cell expansion.
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.
Tokuhira
Michihide
M
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan. tokuhira@saitama-med.ac.jp.
Iizuka
Atsushi
A
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Watanabe
Reiko
R
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Sekiguchi
Naoya
N
Division of Rheumatology, Department of Internal Medicine, Saitama Medical Center, Kawagoe, Saitama, Japan.
Sato
Norihide
N
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Chien
Chen-Kang
CK
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Sekiguchi
Yasunobu
Y
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Nemoto
Tomoe
T
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Hanzawa
Kyoko
K
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Tamaru
Jun-Ichi
JI
Department of Pathology, Saitama Medical Center, Kawagoe, Saitama, Japan.
Itoyama
Shinji
S
Department of Pathology, Saitama Medical Center, Kawagoe, Saitama, Japan.
Suzuki
Hiroshi
H
Transfusion Center, National Defense Medical College, Tokorozawa, Saitama, Japan.
Takeuchi
Tsutomu
T
Division of Rheumatology, Department of Internal Medicine, Saitama Medical Center, Kawagoe, Saitama, Japan.
Mori
Shigehisa
S
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
Kizaki
Masahiro
M
Division of Hematology, Department of Internal Medicine, Saitama Medical Center, 1981 Kamodatsujido, Kawagoe, Saitama, 350-8550, Japan.
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage; D001344:Autopsy; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D020031:Epstein-Barr Virus Infections / Q000134:cerebrospinal fluid / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D007150:Immunohistochemistry; D008099:Liver / Q000276:immunology / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D009206:Myocardium / Q000276:immunology / Q000378:metabolism / Q000473:pathology*; D011241:Prednisone / Q000008:administration & dosage; D012367:RNA, Viral / Q000378:metabolism; D013116:Spinal Cord / Q000276:immunology / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D013154:Spleen / Q000276:immunology / Q000378:metabolism / Q000473:pathology* / Q000821:virology; D014750:Vincristine / Q000008:administration & dosage
D012367:RNA, Viral; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone