Lymphomas arising in immune-privileged sites: insights into biology, diagnosis, and pathogenesis

King, Rebecca; Goodlad, John R.; Calaminici, Maria; Dotlić, Snježana; Montes‐Moreno, Santiago; Oschlies, Ilske; Ponzoni, Maurilio; Traverse-Gléhen, Alexandra; Ott, German; Ferry, Judith A.

doi:10.1007/s00428-019-02698-3

Cited by 67 publications

(78 citation statements)

References 70 publications

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“…Secondary DLBCL is transformed from other less aggressive lymphomas. On the other hand, primary testicular lymphoma can arise from an immune-privileged site, traditionally described as an "immune sanctuary" site [4]. Although primary testicular lymphoma shares its biology with primary DLBCL of the cases are DLBCL with a non-germinal center B-cell (GCB) subtype [5,6].…”

Section: Discussionmentioning

confidence: 99%

Metachronous extranodal natural killer/T-cell lymphoma of nasal type and primary testicular lymphoma

Maeng

Lee

2021

Yeungnam Univ J Med

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A metachronous lymphoma occurs when the second primary lymphoma is diagnosed more than 6 months after the diagnosis of the primary lymphoma. In this article, we report a metachronous extranodal natural killer (NK)/T-cell lymphoma, nasal type, and a primary testicular lymphoma. Extranodal NK/T-cell lymphoma, nasal type, is an extranodal lymphoma of the NK-cell or T-cell lineage, which is prevalent in Asians with a strong association with Epstein-Barr virus (EBV). On the other hand, primary testicular lymphoma is a rare form of extranodal non-Hodgkin lymphoma (NHL), accounting for 1% to 2% of NHL cases [1]. Most cases are diffuse large B-cell lymphoma (DLBCL) with extranodal tropism and frequent relapses. To the best of our knowledge, there is no previous report of metachronous lymphoma, including extranodal NK/T-cell lymphoma, nasal type, and primary testicular DLBCL.

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Section: Discussionmentioning

confidence: 99%

Metachronous extranodal natural killer/T-cell lymphoma of nasal type and primary testicular lymphoma

Maeng

Lee

2021

Yeungnam Univ J Med

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“…Based on the data available in the literature about PBL-SS association [40][41][42][43][44], and the current theories about lymphoma prevalence at immune-privileged sites [45], we can assume that the diagnosis of SS-related lymphoid proliferations, especially when occurring in the breast, currently is very challenging and would probably benefit from wider studies including SS patients with prolonged follow-up (>10 years). Therefore, we may suggest more attentive monitoring for lymphoma development in those SS patients who display higher risk factors (such as palpable purpura, low C4, mixed monoclonal cryoglobulinemia) and to incorporate breast surveillance in such patients.…”

Section: Discussionmentioning

confidence: 99%

Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature

Ingravallo

Maiorano

Moschetta

et al. 2020

JCM

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The association between autoimmune diseases, mostly rheumatoid arthritis, systemic lupus erythematosus, celiac disease and Sjögren syndrome, and lymphoma, has been widely demonstrated by several epidemiologic studies. By a mechanism which has not yet been entirely elucidated, chronic activation/stimulation of the immune system, along with the administration of specific treatments, may lead to the onset of different types of lymphoma in such patients. Specifically, patients affected by Sjögren syndrome may develop lymphomas many years after the original diagnosis. Several epidemiologic, hematologic, and histological features may anticipate the progression from Sjögren syndrome into lymphoma but, to the best of our knowledge, a definite pathogenetic mechanism for such progression is still missing. In fact, while the association between Sjögren syndrome and non-Hodgkin lymphoma, mostly extranodal marginal zone lymphomas and, less often, diffuse large B-cell, is well established, many other variables, such as time of onset, gender predilection, sites of occurrence, subtype of lymphoma, and predictive factors, still remain unclear. We report on a rare case of primary breast lymphoma occurring three years after the diagnosis of Sjögren syndrome in a 57-year-old patient. The diagnostic work-up, including radiograms, core needle biopsy, and histological examination, is discussed, along with emerging data from the recent literature, thus highlighting the usefulness of breast surveillance in Sjögren syndrome patients.

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“…Lymphoma cells do not necessarily have to acquire specific drug-resistant phenotypes to be able to survive anti-lymphoma therapy. A typical example is the involvement of the central nervous system (CNS) or other immune-privileged sites [71]. Thanks to the hemato-encephalic barrier the CNS compartment is not exposed to effective plasma levels of standard front-line anti-lymphoma regimen (e.g., R-CHOP), thereby enabling lymphoma cell survival with no need for large-scale genomic, transcriptional, or post-translational changes.…”

Section: Compartmentalization Of Lymphoma Cells and Survival Of Anti-mentioning

confidence: 99%

Drug Resistance in Non-Hodgkin Lymphomas

Klener

Klánová

2020

IJMS

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Non-Hodgkin lymphomas (NHL) are lymphoid tumors that arise by a complex process of malignant transformation of mature lymphocytes during various stages of differentiation. The WHO classification of NHL recognizes more than 90 nosological units with peculiar pathophysiology and prognosis. Since the end of the 20th century, our increasing knowledge of the molecular biology of lymphoma subtypes led to the identification of novel druggable targets and subsequent testing and clinical approval of novel anti-lymphoma agents, which translated into significant improvement of patients’ outcome. Despite immense progress, our effort to control or even eradicate malignant lymphoma clones has been frequently hampered by the development of drug resistance with ensuing unmet medical need to cope with relapsed or treatment-refractory disease. A better understanding of the molecular mechanisms that underlie inherent or acquired drug resistance might lead to the design of more effective front-line treatment algorithms based on reliable predictive markers or personalized salvage therapy, tailored to overcome resistant clones, by targeting weak spots of lymphoma cells resistant to previous line(s) of therapy. This review focuses on the history and recent advances in our understanding of molecular mechanisms of resistance to genotoxic and targeted agents used in clinical practice for the therapy of NHL.

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Lymphomas arising in immune-privileged sites: insights into biology, diagnosis, and pathogenesis

Cited by 67 publications

References 70 publications

Metachronous extranodal natural killer/T-cell lymphoma of nasal type and primary testicular lymphoma

Metachronous extranodal natural killer/T-cell lymphoma of nasal type and primary testicular lymphoma

Primary Breast Extranodal Marginal Zone Lymphoma in Primary Sjögren Syndrome: Case Presentation and Relevant Literature

Drug Resistance in Non-Hodgkin Lymphomas

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