An immunocompetent patient with a recurrence-free Epstein-Barr virus positive plasmacytoma possesses robust Epstein-Barr virus specific T-cell responses

Chatterjee, Bithi; Sahli, Laura; Chijioke, Obinna; Went, Philip; Münz, Christian; Trojan, Andreas

doi:10.3324/haematol.2017.172791

Cited by 5 publications

(6 citation statements)

References 13 publications

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“…EBER-positive PCN in immunocompetent patients have been reported in few studies with varied incidence, 4–10 likely due to small sample sizes, geographic, or ethnic differences. Two USA studies reported similar EBER positivity (3 of 22, 13.5% and 3 of 20, 15%) in extramedullary plasmacytoma and none of 106 PCM.…”

Section: Discussionmentioning

confidence: 99%

“…2 Epstein-Barr virus (EBV) positivity in PCN has been reported in minor subsets of immunocompetent patients. 4–10 Angiofibroma, characterized by neoplastic fibrovascular proliferation, is another uncommon tumor occurring mostly in the nasopharyngeal area, similar to extramedullary plasmacytoma. 11 Unlike PCN, angiofibroma occurs mainly in adolescent male with an average age of 14 to 17 year-old.…”

Section: Introductionmentioning

confidence: 99%

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EBV-Positive Plasmacytomas Involving a Nasopharyngeal Angiofibroma in an Adolescent

2021

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We report comprehensive characterization of an unusual collision tumor comprising extramedullary plasmacytomas and nasopharyngeal angiofibroma in a pediatric patient, which has yet to be reported. Histologically, the nasopharyngeal angiofibroma showed typical morphology with a diffuse, dense plasmacytic infiltrate in the stroma. The neoplastic plasma cells showed a spectrum of well-differentiated, plasmablastic, and anaplastic morphology, Epstein-Barr virus encoded RNA (EBER) positivity, and aberrant immunophenotype. Fluorescence in situ hybridization using a plasma cell myeloma targeted panel detected gains of 1q21.3 ( CKS1B, x3), 3q27 ( BCL6, x4), and 11q22.3 ( ATM, x3), but no rearrangement of ALK and MYC. A 50-gene next generation sequencing lymphoma panel failed to detect any pathogenic mutation. Plasmacytoma with EBER positivity and plasmablastic morphology must be distinguished from plasmablastic lymphoma due to different clinical management and prognosis. This case highlights the importance of a thorough pathological evaluation of a mass lesion with synchronous neoplastic processes.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

EBV-Positive Plasmacytomas Involving a Nasopharyngeal Angiofibroma in an Adolescent

2021

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“… 2016 ), suggesting that EBV-associated plasmacytomas are very efficiently controlled by the immune system (Chatterjee et al. 2017 ). In contrast, monogenic primary immunodeficiencies have been characterized that predispose either for isolated KS development or more generally for susceptibility to infectious diseases, including KS (Table 2 ).…”

Section: Immunodeficiencies That Predispose For Ksmentioning

confidence: 99%

Immunodeficiencies that predispose to pathologies by human oncogenic γ-herpesviruses

Damania

Münz

2019

FEMS Microbiology Reviews

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Human γ-herpesviruses include the closely related tumor viruses Epstein Barr virus (EBV) and Kaposi sarcoma-associated herpesvirus (KSHV). EBV is the most growth-transforming pathogen known and is linked to at least seven human malignancies. KSHV is also associated with three human cancers. Most EBV- and KSHV-infected individuals fortunately remain disease-free despite persistent infection and this is likely due to the robustness of the immune control that they mount against these tumor viruses. However, upon immune suppression EBV- and KSHV-associated malignancies emerge at increased frequencies. Moreover, primary immunodeficiencies with individual mutations that predispose to EBV or KSHV disease allow us to gain insights into a catalog of molecules that are required for the immune control of these tumor viruses. Curiously, there is little overlap between the mutation targets that predispose individuals to EBV versus KSHV disease, even so both viruses can infect the same host cell, human B cells. These differences will be discussed in this review. A better understanding of the crucial components in the near-perfect life-long immune control of EBV and KSHV should allow us to target malignancies that are associated with these viruses, but also induce similar immune responses against other tumors.

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“…Moreover, EBV-positive PCN was mainly reported in immunodeficient patients, while only a few cases occurred in immunocompetent individuals 1–20 . This leaves open the question whether the prevalence of EBV in PCN varies according to the histologic subtype and tumor location in immunocompetent patients.…”

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confidence: 99%

Epstein-Barr Virus–Positive Plasma Cell Neoplasms in Immunocompetent Patients

Xiao,

Lin,

et al. 2023

American Journal of Surgical Pathology

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Epstein-Barr virus (EBV)-positive plasma cell neoplasms (PCNs) in immunocompetent patients are a rare entity, the clinicopathological and prognostic features of which have not been well characterized. Fifteen cases of EBV-positive PCN arising in immunocompetent patients from south China were retrospectively analyzed, and an additional 44 cases from the literature were reviewed. The overall EBV-positive rate defined by EBV-encoded small RNAs (EBERs) in-situ hybridization of PCNs was 12.3% (15/122), and it was significantly higher in plasmacytoma (17.1%, 13/76) than in plasma cell myeloma/multiple myeloma (4.3%, 2/46; P=0.031). The age of the patients ranged from 17 to 79 years, with a median age of 56 years. There was a large preponderance of men, with a male-to-female ratio of 4:1. Solitary plasmacytoma of bone (23.8%, 5/21) had comparable EBV-encoded small RNAs-positive rates with extramedullary plasmacytoma arising in the upper respiratory tract (19.5%, 8/41; P=0.949). Anaplastic and classic cytologic appearance was observed in 61.5% (8/13) and 38.5% (5/13) of EBV-positive plasmacytomas, respectively. Cases with an anaplastic cytologic appearance had a significantly higher Ki-67 proliferation index than those with a classic cytologic appearance (median: 55% vs. 10%, P=0.001). In the combined cohorts, anaplastic/plasmablastic cytologic appearance was significantly more common in extramedullary plasmacytoma arising in the upper respiratory tract (72.0%, 18/25) than outside the upper respiratory tract (11.1%, 1/9; P=0.006). Among the 59 cases of EBV-positive PCN, survival data of 34 cases were available for analysis, including 30 cases of plasmacytoma and 4 cases of plasma cell myeloma/multiple myeloma. There was no statistically significant difference in overall survival between patients with EBV-positive plasmacytomas in the combined cohorts and EBV-negative plasmacytomas in the present cohort. The prevalence of EBV in PCN in immunocompetent patients varies according to histologic subtype and tumor location. Compared with EBV-negative cases, EBV-positive plasmacytomas tend to have an anaplastic/plasmablastic cytologic appearance. No significant impact of EBV infection on clinical outcomes is observed in the limited number of reported cases.

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An immunocompetent patient with a recurrence-free Epstein-Barr virus positive plasmacytoma possesses robust Epstein-Barr virus specific T-cell responses

Cited by 5 publications

References 13 publications

EBV-Positive Plasmacytomas Involving a Nasopharyngeal Angiofibroma in an Adolescent

EBV-Positive Plasmacytomas Involving a Nasopharyngeal Angiofibroma in an Adolescent

Immunodeficiencies that predispose to pathologies by human oncogenic γ-herpesviruses

Epstein-Barr Virus–Positive Plasma Cell Neoplasms in Immunocompetent Patients

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