Fulminant Epstein-Barr Virus-associated T-cell Lymphoproliferative Disorder in an Immunocompetent Middle-aged Man Presenting with Chronic Diarrhea and Gastrointestinal Bleeding.
10.3346/jkms.2011.26.8.1103
- Author:
Jamshid ABDUL-GHAFAR
1
;
Jae Woo KIM
;
Kwang Hwa PARK
;
Mee Yon CHO
Author Information
1. Department of Pathology, Yonsei University Wonju College of Medicine, Wonju, Korea. meeyon@yonsei.ac.kr
- Publication Type:Case Report
- Keywords:
Epstein-Bar Virus Infections;
Lymphoproliferatife Disorders;
Atypical T-cell Proliferation
- MeSH:
Chronic Disease;
Colonoscopy;
Diarrhea/*diagnosis;
Disseminated Intravascular Coagulation/diagnosis;
Epstein-Barr Virus Infections/complications/virology;
Feces/virology;
Gastrointestinal Hemorrhage;
Herpesvirus 4, Human/genetics/*isolation & purification;
Humans;
Lymphoproliferative Disorders/*diagnosis/immunology/virology;
Male;
Middle Aged;
RNA, Viral/analysis;
T-Lymphocytes/*immunology/pathology
- From:Journal of Korean Medical Science
2011;26(8):1103-1107
- CountryRepublic of Korea
- Language:English
-
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.
