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

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Nobuyoshi Hanaoka *
Shogo Murata
Hiroki Hosoi
Aiko Shimokado
Toshiki Mushino
Kodai Kuriyama
Kazuo Hatanaka
Akinori Nishikawa
Miwa Kurimoto
Takashi Sonoki
Yasuteru Muragaki
Hideki Nakakuma
(*) Corresponding Author:
Nobuyoshi Hanaoka | nhanaoka@wakayama-med.ac.jp


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.


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