Frequent occurrence of B‐cell lymphomas in angioimmunoblastic T‐cell lymphoma and proliferation of Epstein–Barr virus‐infected cells in early cases

Abstract

Summary Secondary lymphomas occurring in the setting of angioimmunoblastic T‐cell lymphoma (AILT) are considered to be rare. Their occurrence has been attributed to Epstein–Barr virus (EBV)‐associated lymphoproliferations. A previous study detected a dysregulated hypermutation process in B‐cells of AILT. The present study aimed at estimating the frequency of B‐cell lymphomas in AILT. By studying the expression of EBV and activation‐induced cytidine deaminase (AID) as an indicator of hypermutating cells, we assessed whether B‐cell lymphoproliferations in AILT were strictly associated with EBV and whether hypermutation might contribute to lymphomagenesis. Among 161 cases of AILT, diagnosed between 1996 and 2005 at the lymph node registry, Frankfurt, Germany, 19 cases were detected that also had B‐cell non‐Hodgkin lymphoma (NHL) and two cases had classical Hodgkin lymphoma (HL). EBV was detected in tumour cells of 7/18 NHL and both HL, suggesting that factors other than EBV contribute to lymphomagenesis. AID was expressed in AILT in large cells disseminated in the tissue, implying that the process of somatic hypermutation is ongoing in AILT, although the GC architecture is disrupted. This might be relevant in the development of secondary lymphomas.

Small Bowel Perforation Caused by Epstein-Barr Virus-Associated B Cell Lymphoma in a Patient with Angioimmunoblastic T-Cell Lymphoma

“…[7][8][9][10][11][12][13][14][15] It is commonly considered that EBV-associated secondary B cell lymphomas in AITL develop from EBV-infected B cells in lymph nodes. However, EBV-infected B cells were not detected by EBER-ISH before development of secondary lymphoma in our case.…”

“…However, relatively few cases of EBV-associated B cell lymphomas in AITL have been fully described so far. [7][8][9][10][11][12][13][14][15] In a previous large study, only three cases out of more than 170 cases with AITL developed EBV-associated secondary B cell lymphomas. 10 We report here a rare case of AITL, in which small bowel perforation was thought to be caused by EBV-associated B cell lymphoma after combination chemotherapy and high dose corticosteroid treatment, and discuss the etiology of the secondary B cell lymphoma with a review of the literature.…”

Epstein–Barr virus-associated B-cell lymphoma secondary to FCD-C therapy in patients with peripheral T-cell lymphoma

“…EBVinfected B-cells have been described in most cases of AILT as non-neoplastic bystander cells [6,7]. However, recently a few cases of EBV-associated B-cell lymphomas arising in patients with T-cell lymphoma have been reported, most commonly in patients with AILT [5,8].…”

Recent Progress in the Understanding of Angioimmunoblastic T-cell Lymphoma

“…Nine of 20 patients (45%) were positive for EBV, while 11 (55%) were negative for EBV. 70 These data suggest that although EBV may take part in the development of B-cell lymphomas in the majority of cases, it does not explain a substantial proportion, as some were negative for EBV.…”