title: Fulminant Epstein-Barr virus-associated T-cell lymphoproliferative disorder in an immunocompetent middle-aged man presenting with chronic diarrhea and gastrointestinal bleeding.
abstract: The World Health Organization (WHO) recently defined systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorders (LPD) of childhood as a life-threatening illness. However, this rare disease has not been extensively studied. Here we report a case of systemic EBV-positive T-cell LPD in a previously healthy middle-aged man with a chief complaint of chronic diarrhea. The initial colon biopsy showed focal infiltration of EBV-positive small lymphocytes without any atypia. However, the disease rapidly progressed and the patient required a total colectomy due to severe gastrointestinal bleeding. Three and half months after admission, the patient died from a complication of disseminated intravascular coagulation. The resected colon showed diffuse infiltration of EBV-positive atypical lymphocytes with ischemic change. Most atypical lymphocytes were CD3+ or CD5+. The monoclonality of EBV was demonstrated by sequence variation analysis of the latent membrane protein 1 (LMP1) gene in the colectomy specimen as well as in the initial biopsy.
authors.affiliation: Department of Pathology, Yonsei University Wonju College of Medicine, Wonju, Korea.
authors.forename: Jamshid
authors.initials: J
authors.lastname: Abdul-Ghafar
authors.forename: Jae Woo
authors.initials: JW
authors.lastname: Kim
authors.forename: Kwang Hwa
authors.initials: KH
authors.lastname: Park
authors.forename: Mee-Yon
authors.initials: MY
authors.lastname: Cho
mesh_terms: D002908:Chronic Disease; D003113:Colonoscopy; D003967:Diarrhea / Q000175:diagnosis*; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis; D020031:Epstein-Barr Virus Infections / Q000150:complications / Q000821:virology; D005243:Feces / Q000821:virology; D006471:Gastrointestinal Hemorrhage; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000175:diagnosis* / Q000276:immunology / Q000821:virology; D008297:Male; D008875:Middle Aged; D012367:RNA, Viral / Q000032:analysis; D013601:T-Lymphocytes / Q000276:immunology* / Q000473:pathology
chemical_list: D012367:RNA, Viral