Skip to main content
Have a personal or library account? Click to login
Primary Cutaneous Marginal Zone B-Cell Lymphoma Complicating Long-Standing Primary Sjögren’s Syndrome – A Multidisciplinary Diagnostic Challenge Cover

Primary Cutaneous Marginal Zone B-Cell Lymphoma Complicating Long-Standing Primary Sjögren’s Syndrome – A Multidisciplinary Diagnostic Challenge

Open Access
|Aug 2026

References

  1. Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, et al. Sjögren syndrome. Nat Rev Dis Primers. 2016;2:16047..
  2. Nocturne G, Mariette X. B cells in the pathogenesis of primary Sjögren syndrome. Nat Rev Rheumatol. 2018;14(3):133-45.
  3. Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjögren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006;65(6):796-803.
  4. Ekström Smedby K, Vajdic CM, Falster M, Engels EA, Martínez-Maza O, Turner J, et al. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. Blood. 2008;111(8):4029-38.
  5. Alunno A, Leone MC, Giacomelli R, Gerli R, Carubbi F. Lymphoma and lymphomagenesis in primary Sjögren’s syndrome. Front Med (Lausanne). 2018;5:102.
  6. Stergiou IE, Poulaki A, Voulgarelis M. Pathogenetic mechanisms implicated in Sjögren’s syndrome lymphomagenesis: a review of the literature. J Clin Med. 2020;9(12):3794.
  7. Mohammadnezhad L, Shekarkar Azgomi M, La Manna MP, Guggino G, Botta C, Dieli F, et al. B-cell receptor signaling is thought to be a bridge between primary Sjogren syndrome and diffuse large B-cell lymphoma. Int J Mol Sci. 2023;24(9):8385.
  8. Cheah CY, Seymour JF. Marginal zone lymphoma: 2023 update on diagnosis and management. Am J Hematol. 2023;98(10):1645-57.
  9. Alaggio R, Amador C, Anagnostopoulos I, Attygalle AD, Araujo IBO, Berti E, et al. The 5th edition of the World Health Organization classification of haematolymphoid tumours: lymphoid neoplasms. Leukemia. 2022;36(7):1720-48.
  10. Campo E, Jaffe ES, Cook JR, Quintanilla-Martinez L, Swerdlow SH, Anderson KC, et al. The International Consensus Classification of Mature Lymphoid Neoplasms: a report from the Clinical Advisory Committee. Blood. 2022;140(11):1229-53.
  11. Willemze R, Cerroni L, Kempf W, Berti E, Facchetti F, Swerdlow SH, et al. The 2018 update of the WHOEORTC classification for primary cutaneous lymphomas. Blood. 2019;133(16):1703-14.
  12. Bernardelli A, Carazzai E, Bugnotto B, Bellinato F, Krampera M, Visco C. Primary cutaneous B-cell lymphomas: an updated portrait of classification, biology, and clinical management. Eur J Haematol. 2026;116 (2):116-28.
  13. Dumont M, Battistella M, Ram-Wolff C, Bagot M, de Masson A. Diagnosis and treatment of primary cutaneous B-cell lymphomas: state of the art and perspectives. Cancers (Basel). 2020;12(6):1497.
  14. Gibson SE, Swerdlow SH. How I diagnose primary cutaneous marginal zone lymphoma. Am J Clin Pathol. 2020;154(4):428-49.
  15. Olsen EA, Whittaker S, Willemze R, Pinter-Brown L, Foss F, Geskin L, et al. Primary cutaneous lymphoma: recommendations for clinical trial design and staging update from the ISCL, USCLC, and EORTC. Blood. 2022;140(5):419-37.
  16. Qin Y, Wang Y. Penicillin in Borrelia burgdorferi-negative primary cutaneous marginal zone lymphoma: a case series and literature review. Acta Derm Venereol. 2022;102:adv00668.
DOI: https://doi.org/10.2478/sjdv-2026-0005 | Journal eISSN: 2406-0631 | Journal ISSN: 1821-0902
Language: English
Page range: 32 - 42
Submitted on: Jun 2, 2026
Accepted on: Jun 10, 2026
Published on: Aug 10, 2026
Published by: Serbian Association of Dermatovenereologists (SAD)
In partnership with: Paradigm Publishing Services
Publication frequency: 4 issues per year

© 2026 Andrijana Kovačević, Kristina Andrić, Vesna Miličić, Gordana Ristić, Ana Ravić Nikolić, published by Serbian Association of Dermatovenereologists (SAD)
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.