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Lymphomas in Patients With Sjögren's Syndrome Are Marginal Zone B-Cell Neoplasms, Arise in Diverse Extranodal and Nodal Sites, and Are Not Associated With Viruses
Open accessAbstract
The occurrence of non-Hodgkin's lymphoma (NHL) is the most serious complication of Sjögren's syndrome (SS). We performed a study of 16 NHLs occurring in patients with an underlying SS. These lymphomas arose not only in salivary glands (7 cases) but also in other mucosal extranodal sites (the stomach [4 cases], the lung [3 cases], the skin [3 cases], the buccal mucosa [1 case], the thymus [1 case]) and in nodal sites (8 cases). Low-grade marginal zone lymphomas (MZL) were diagnosed in 12 of the 16 patients, 9 of mucosa-associated lymphoid tissues (MALT) type in mucosal sites and 3 exclusively nodal. The 4 other patients presented with a high-grade B-cell lymphoma that was probably a histological transformation of an underlying low-grade MZL at least in 3 of the cases involving skin, stomach, and parotid, respectively. A t(14; 18) translocation was detected in 1 of 8 lymphomas tested. We detected serum anti-p53 antibodies in 2 of the 14 studied patients. p53 protein was detected in 1 of 11 lymphomas tested. LMP protein and Eber RNAs of Epstein-Barr virus (EBV) were not detected in the 16 NHL biopsies. Using polymerase chain reaction, EBV was never detected except in 1 of 4 parotid lymphomas. No human T-lymphotropic virus 1 or human herpes virus 8 DNAs were detected in NHL biopsies. None of the patients had hepatitis C virus infection found using serological methods. Chemotherapy was usually efficient. In conclusion, lymphomas occurring in patients with an underlying SS are in most cases MZL. These lymphomas are not associated with viruses known to be present in other types of lymphomas. Some of the translocations or mutations of oncogenes or antioncogenes described in other lymphomas are detected in SS-associated lymphomas.
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Citations
Cited by
Mariette, X. (2002) · Blood · doi:10.1182/blood.v99.11.3909 · Cited by 428
Self-citationFox, R. I., Michelson, P., Casiano, C. A., Hayashi, J., & Stern, M. (2000) · Clinics in Dermatology · doi:10.1016/s0738-081x(00)00135-8 · Cited by 355
Xerostomia: Clinical Aspects and Treatment
Cassolato, S. F., & Turnbull, R. S. (2003) · Gerodontology · doi:10.1111/j.1741-2358.2003.00064.x · Cited by 349
Sjögren Syndrome‐associated lymphomas: an update on pathogenesis and management
Nocturne, G., & Mariette, X. (2015) · British Journal of Haematology · doi:10.1111/bjh.13192 · Cited by 295
Self-citationBaimpa, E., Dahabreh, I. J., Voulgarelis, M., & Moutsopoulos, H. M. (2009) · Medicine · doi:10.1097/md.0b013e3181b76ab5 · Cited by 276
Metadata
- Authors
- Royer, B., Cazals-Hatem, D., Sibilia, J., Agbalika, F., Cayuela, J.-M., Soussi, T., Maloisel, F., Clauvel, J.-P., Brouet, J.-C., & Mariette, X.
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 1997-07-15
- Volume
- 90
- Issue
- 2
- Pages
- 766-775
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W140424242
- Cited by
- 348
- References
- 0
- FWCI
- 7.32
- Type
- journal article
- Language
- en
- Open access
- bronze
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- CoChat. (2026). Citation check: Lymphomas in Patients With Sjögren's Syndrome Are Marginal Zone B-Cell Neoplasms, Arise in Diverse Extranodal and Nodal Sites, and Are Not Associated With Viruses. https://app.cochat.ai/papers/10.1182/blood.v90.2.766
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- Royer, B., Cazals-Hatem, D., Sibilia, J., Agbalika, F., Cayuela, J.-M., Soussi, T., Maloisel, F., Clauvel, J.-P., Brouet, J.-C., & Mariette, X. (1997). Lymphomas in Patients With Sjögren's Syndrome Are Marginal Zone B-Cell Neoplasms, Arise in Diverse Extranodal and Nodal Sites, and Are Not Associated With Viruses. Blood. https://doi.org/10.1182/blood.v90.2.766
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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