title: Young adult onset systemic Epstein-Barr virus-positive T-cell lymphoproliferative disorders of childhood.
abstract: A 20-year-old woman had a fever, pancytopenia, and liver failure, and was suspected to be suffering from chronic active Epstein-Barr virus (EBV) infection, based on the detection of high EBV-DNA and EBV antibody titers at another hospital. At our institution one month later, clinical manifestations had diminished, and antibody titers had decreased but remained elevated relative to normal levels. Four days later, the patient required hospitalization due to fever, liver damage, and cervical lymphadenopathy. Bone marrow examination and lymph node biopsy results showed EBV-positive cytotoxic T-cells that were predominantly CD4-positive. The disease followed a fulminant course and the patient died of multiple organ failure on hospitalization day 11. Because complicated chromosomal aberrations and T-cell receptor gene rearrangements were identified, we diagnosed her as having systemic EBV-positive T-cell lymphoproliferative disorder of childhood. This disease type includes a lymphoproliferative disorder that is associated with chronic active EBV infection. However, it is clinically different from the type following acute EBV infection. We consider distinguishing between these two types to be important for selecting an early diagnostic procedure and the optimal therapy.
authors.affiliation: Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University.
authors.forename: Mizu
authors.initials: M
authors.lastname: Sakai
authors.forename: Kazuto
authors.initials: K
authors.lastname: Togitani
authors.forename: Tsukie
authors.initials: T
authors.lastname: Tsukuda
authors.forename: Yu
authors.initials: Y
authors.lastname: Arakawa
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ikezoe
authors.forename: Makoto
authors.initials: M
authors.lastname: Hiroi
authors.forename: Akihito
authors.initials: A
authors.lastname: Yokoyama
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D017809:Fatal Outcome; D005260:Female; D004854:Herpesvirus 4, Human; D006801:Humans; D016399:Lymphoma, T-Cell / Q000188:drug therapy / Q000473:pathology* / Q000821:virology; D055815:Young Adult