CD4-CD8- T-cell polymyositis in a patient with chronic active Epstein-Barr virus infection.
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.
Tsutsumi
Sachiko
S
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan.
Ohga
Shouichi
S
Nomura
Akihiko
A
Takada
Hidetoshi
H
Sakai
Shuji
S
Ohshima
Koichi
K
Sumimoto
Kotoko
K
Hara
Toshiro
T
D000293:Adolescent; D015496:CD4-Positive T-Lymphocytes / Q000473:pathology*; D018414:CD8-Positive T-Lymphocytes / Q000473:pathology*; D002908:Chronic Disease; D020031:Epstein-Barr Virus Infections; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D017285:Polymyositis / Q000097:blood* / Q000175:diagnosis / Q000473:pathology / Q000821:virology*