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).
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: firstname.lastname@example.org