B-cell-rich T-cell lymphoma associated with Epstein-Barr virus-reactivation and T-cell suppression following antithymocyte globulin therapy in a patient with severe aplastic anemia

• Main Authors: Nobuyoshi Hanaoka, Shogo Murata, Hiroki Hosoi, Aiko Shimokado, Toshiki Mushino, Kodai Kuriyama, Kazuo Hatanaka, Akinori Nishikawa, Miwa Kurimoto, Takashi Sonoki, Yasuteru Muragaki, Hideki Nakakuma
• Format: Article
• Language: English
• Published: PAGEPress Publications 2015-09-01
• Series: Hematology Reports
• Subjects: Lymphoproliferative disorder; Epstein-Barr virus; molecular autopsy; anti-thymocyte globulin; aplastic anemia
• Online Access: http://www.pagepress.org/journals/index.php/hr/article/view/5906
• ISSN: 2038-8322; 2038-8330

B-cell lymphoproliferative disorder (B-LPD) is generally characterized by the proliferation of Epstein-Barr virus (EBV-infected B lymphocytes. We here report the development of EBV-negative B-LPD associated with EBV-reactivation following antithymocyte globulin (ATG) therapy in a patient with aplastic anemia. The molecular autopsy study showed the sparse EBV-infected clonal T cells could be critically involved in the pathogenesis of EBV-negative oligoclonal B-LPD through cytokine amplification and escape from T-cell surveillances attributable to ATG-based immunosuppressive therapy, leading to an extremely rare B-cell-rich T-cell lymphoma. This report helps in elucidating the complex pathophysiology of intractable B-LPD refractory to rituximab.