Autopsy Case of Epstein-Barr Virus-associated T-cell Post-transplant Lymphoproliferative Disorder with Mono- and Polymorphic Lesions: Possibility of 'Polymorphic T-cell Post-transplant Lymphoproliferative Disorder'

• Published in: Internal Medicine Vol. 64; no. 3; pp. 439 - 447
• Main Authors: Sato, Keijiro, Ueki, Toshimitsu, Tokutake, Takayoshi, Watanabe, Masahide, Shigeto, Shohei, Kanno, Hiroyuki, Sumi, Masahiko, Kobayashi, Hikaru
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01.02.2025; Japan Science and Technology Agency
• Subjects: Autopsy; Case Report; Cell proliferation; Epstein-Barr virus; Epstein-Barr Virus Infections - complications; Epstein-Barr Virus Infections - immunology; Epstein-Barr Virus Infections - pathology; Fatal Outcome; hemophagocytic syndrome; Herpesvirus 4, Human; Humans; Ileum; Immunoproliferative diseases; Lymphocytes; Lymphocytes T; Lymphoproliferative Disorders - diagnosis; Lymphoproliferative Disorders - etiology; Lymphoproliferative Disorders - immunology; Lymphoproliferative Disorders - pathology; Lymphoproliferative Disorders - virology; Male; Middle Aged; Polymerase chain reaction; polymorphic; post-transplant lymphoproliferative disorder; Postoperative Complications - pathology; Posttransplant lymphoproliferative disorders; T cell; T cell receptors; T-Lymphocytes - immunology; T-Lymphocytes - pathology; T-Lymphocytes - virology; polymorphic; Epstein-Barr virus; post-transplant lymphoproliferative disorder; T cell; hemophagocytic syndrome
• ISSN: 0918-2918; 1349-7235; 1349-7235
• DOI: 10.2169/internalmedicine.3484-24

Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disease (PTLD) is predominantly of B cell origin. The concept of clonal evolution from poly- to monoclonal lymphoproliferation has been put forward, but T-cell PTLDs are rare with an unknown etiology. In a unique autopsy case of a 53-year-old man with EBV-associated T-cell PTLD, we observed polymorphic T-cell proliferation across several organs and monomorphic T-cell proliferation in the perforated ileum. Interestingly, both manifestations exhibited identical monoclonal peaks in the T-cell receptor rearrangement polymerase chain reaction (PCR) analyses. These findings suggest the existence of clonal evolution in EBV-associated T-cell PTLD, leading to the proposal of the novel concept of polymorphic T-cell PTLD.