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| IJCEP Copyright © 2007-All rights reserved. |
| International Journal of Clinical and Experimental Pathology |
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
Full Text PDF
Address all correspondences 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
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
Full Text PDF
Address all correspondences 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