title: Epstein-Barr virus-positive cytotoxic T-cell lymphoma followed by chronic active Epstein-Barr virus infection-associated T/NK-cell lymphoproliferative disorder: a case report.
abstract: A 30-year-old female patient presented with intestinal Epstein-Barr virus (EBV)-positive cytotoxic T-cell lymphoma (EBV+ CTL), which was surgically resected. Fourteen years later, she returned to our hospital with hypersensitivity to mosquito bites and was diagnosed with chronic active EBV infection-associated T/NK-cell lymphoproliferative disorder (CAEBV/TNK-LPD). She developed systemic EBV+ CTL at age 47 years during the 2.5-year clinical course of CAEBV/TNK-LPD, despite multiagent chemotherapy and allogeneic stem cell transplantation. Afterward, she had a rapidly deteriorating clinical course and died at age 48 years. The immunophenotype of the EBV+ CTL was consistently a CD3, CD8, and cytotoxic molecule-positive type with the same clonality in polymerase chain reaction analysis of T-cell receptor-γ chain gene rearrangement. This is the first reported case of EBV+ CTL preceding the clinical presentation of CAEBV/TNK-LPD. The present case was unique in suggesting a close relationship between EBV+ CTL and chronic active EBV infection.
authors.affiliation: Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya 466-8560, Japan. Electronic address: katopath@med.nagoya-u.ac.jp.
authors.forename: Seiichi
authors.initials: S
authors.lastname: Kato
authors.forename: Tomoko
authors.initials: T
authors.lastname: Miyata
authors.forename: Katsuyoshi
authors.initials: K
authors.lastname: Takata
authors.forename: Satoko
authors.initials: S
authors.lastname: Shimada
authors.forename: Yoshinori
authors.initials: Y
authors.lastname: Ito
authors.forename: Akihiro
authors.initials: A
authors.lastname: Tomita
authors.forename: Ahmed Ali
authors.initials: AA
authors.lastname: Elsayed
authors.forename: Emiko
authors.initials: E
authors.lastname: Takahashi
authors.forename: Naoko
authors.initials: N
authors.lastname: Asano
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Kinoshita
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kimura
authors.forename: Shigeo
authors.initials: S
authors.lastname: Nakamura
mesh_terms: D000328:Adult; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000473:pathology* / Q000821:virology; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification*; D006801:Humans; D016399:Lymphoma, T-Cell / Q000150:complications / Q000473:pathology* / Q000821:virology; D008232:Lymphoproliferative Disorders / Q000150:complications / Q000473:pathology* / Q000821:virology; D013602:T-Lymphocytes, Cytotoxic / Q000473:pathology* / Q000821:virology
keywords: Chronic active Epstein-Barr virus infection; Cytotoxic molecule; Epstein-Barr virus–associated T/NK-cell lymphoproliferative disorders; T-cell lymphoma