Cerebellar EBV-associated diffuse large B cell lymphoma following angioimmunoblastic T cell lymphoma

Abstract

Epstein-Barr virus (EBV)-associated B cell lymphoproliferative disorders may be seen in patients with angioimmunoblastic T cell lymphoma (AITL). Although both nodal and extranodal sites of involvement have been described, central nervous system involvement by B cell lymphoma following AITL has not previously been documented. We report a first example of such unusual presentation, in which an 80-year-old man developed diffuse large B cell lymphoma (DLBCL) in the cerebellum 4 months after the initial diagnosis of AITL. EBV-encoded RNAs were detected in the DLBCL, suggesting that EBV played a pivotal role in the pathogenesis of high-grade histologic progression of AITL. The patient survived less than 9 months after his initial diagnosis of AITL. We believe that this case expands the spectrum of extranodal manifestation of EBV-positive B cell lymphoma associated with AITL and illustrates the importance of recognition of this association when encountering unusual central nervous system lesions in patients with known AITL.

Bone Marrow Involvement of Epstein-Barr Virus-Positive Large B-Cell Lymphoma in a Patient with Angioimmunoblastic T-Cell Lymphoma

“…However, clonal B-cell proliferation, such as large B-cell lymphoma or plasma cell myeloma, is a rare event in AITL. Previously, only lymph nodal [ 6 ], tonsillar [ 7 ], cutaneous [ 2 ], upper leg [ 2 ], or cerebellar [ 8 ] involvement of large B-cell lymphoma accompanying AITL has been reported. To the best of our knowledge, this is the first report of diffuse BM infiltration in large B-cell lymphoma with AITL.…”

Overlap between EBV‐positive diffuse large B‐cell lymphoma, NOS, in a young patient and angioimmunoblastic T‐cell lymphoma: A diagnostic pitfall

“…In patients with established AITL, high-grade histologic progression, when occurring on infrequent occasions, is almost always to EBV + DLBCL (AITL progressing to EBV + DLBCL). 4 Simultaneous presentation of EBV + DLBCL and AITL in the same anatomical site (composite AITL/B-cell lymphoma) is also described. 5 While the pathogenesis underlying progression to DLBCL in the context of AITL has not been fully elucidated, hypothetical models proposed by Dunleavy et al suggest CXCL13 mediated co-stimulatory loop of EBV-infected B cells and T cells.…”

“…In patients with established AITL, high‐grade histologic progression, when occurring on infrequent occasions, is almost always to EBV + DLBCL (AITL progressing to EBV + DLBCL) 4 . Simultaneous presentation of EBV + DLBCL and AITL in the same anatomical site (composite AITL/B‐cell lymphoma) is also described 5 .…”

Epstein–Barr Virus Positive Diffuse Large B-Cell Lymphoma Transformed Into Angioimmunoblastic T-Cell Lymphoma After Treatment: A Case Report and Literature Review

“…EBV positive DLBCL patients following AITL have been reported in some case report [3][4][5][6] . EBV infection may reduce the immune function of AITL patient, and lead to B cell dysfunction.…”