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Int J Clin Exp Pathol 1(2):185-197;2008

Case Report
Fulminant EBV-driven CD8 T-cell Lymphoproliferative Disorder Following Primary Acute
EBV Infection: A Unique Spectrum of T-Cell Malignancy

Ken H. Young, Dahua Zhang, Jeffery T. Malik and Eliot C. Williams

Departments of 1Pathology and Laboratory Medicine, 2Medicine and Hematology, University of Wisconsin Hospital and Clinics, University of
Wisconsin School of Medicine and Public Health, Madison, Wisconsin.

Received 10 July 2007; accepted 16 July 2007; available online 1 January 2008

Abstract: Fulminant Epstein-Barr virus (EBV)-driven clonal T-cell lymphoproliferative disorder (T-LPD) is rare and most patients are of Asian
origin. The disease usually develops shortly after primary acute EBV infection and the mechanism remains poorly understood. Here we report
such a rare case in a 28-year-old Caucasian female with systemic lupus erythematosus (SLE). Immunophenotypic and molecular studies
revealed that the proliferating lymphoid cells displayed a CD8+ T-cell phenotype with clonal rearrangement of the T-cell receptor gamma gene.
Epstein-Barr virus-encoded RNA was also observed in the clonal lymphoid cells by in situ hybridization. The patient subsequently developed
fatal virus-associated hemophagocytic syndrome one month after the primary acute EBV infection. The case represents the first report of
fulminant EBV-driven CD8+ T-LPD occurring in an immunocompromised Caucasian SLE patient. This study, along with studies of similar
Asian cases reported in the literature, suggests that dysregulated immunity due to either acquired or genetically determined susceptibility may
result in an abnormal response to primary EBV infection and contribute to the pathogenesis of EBV-mediated fatal T-LPD. (IJCEP707010).

Key Words: Fatal infectious mononuleosis, Epstein-Barr virus, T-cell lymphoproliferative disorder, virus-associated hemophagocytic
syndrome, hemophagocytosis, systemic lupus erythematosus

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Address all correspondence to:  Ken H. Young, MD, PhD, Department of Pathology & Laboratory Medicine University of Wisconsin School of
Medicine and Public Health University of Wisconsin Hospital & Clinics 600 N. Highland Avenue, B4-263 Madison, WI 53792-2472; Tel:
608-262-7254; Fax: 608-263-1568; Email:
khyoung@wisc.edu