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

International Journal of Clinical and Experimental Pathology

Eliot C Williams¹  Dahua Zhang¹  Jeffery T Malik¹  Ken H Young¹ 

关键词: Fatal infectious mononuleosis;    Epstein-Barr virus;    T-cell lymphoproliferative disorder;    virus-associated hemophagocytic syndrome;    hemophagocytosis;    systemic lupus erythematosus;   

DOI  :  

学科分类：生理学与病理学

来源: e-Century Publishing Corporation

【 摘 要 】

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.

【 授权许可】

Unknown