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KMID : 0191120110260081103
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Journal of Korean Medical Science
2011 Volume.26 No. 8 p.1103 ~ p.1107
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Fulminant Epstein-Barr Virus-associated T-cell Lymphoproliferative Disorder in an Immunocompetent Middle-aged Man Presenting with Chronic Diarrhea and Gastrointestinal Bleeding
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Abdul Ghafar Jamshid
Kim Jae-Woo
Park Kwang-Hwa
Cho Mee-Yon
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Abstract
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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.
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KEYWORD
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Epstein-Bar Virus Infections, Lymphoproliferatife Disorders, Atypical T-cell Proliferation
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