Онкогематология (Nov 2022)

Lymphoproliferative diseases in primary Sjogren's syndrome

  • V. i. Vasiljev,
  • N. A. Probatova,
  • N. N. Tupitsyn,
  • E. Y. Varlamova,
  • O. A. Logvinenko,
  • A. M. Kovrigina,
  • E. M. Sholohova,
  • M. V. Simonova,
  • T. N. Safonova,
  • V. R. Gorodetsky,
  • N. V. Kokosadze,
  • A. M. Pavlovskaya,
  • T. N. Kondratjeva,
  • Z. G. Kadagidze,
  • E. V. Gayduk

DOI
https://doi.org/10.17650/1818-8346-2007-0-3-16-26
Journal volume & issue
Vol. 0, no. 3
pp. 16 – 26

Abstract

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During long term follow up (median 10 years) of 412 patients with primary Sjogren's syndrome (pSS) 46; (11,2%) women developed non-Hodgkin's lymphoma (NHL). Median pSS duration before development of NHL was 17 years. 43 (93,3%) patients had B-cell and 3 (6,7%) - T-cell NHL. All types of NHL were present except precursor cell lymphomas. Diffuse large B-cell lymphoma (LBCL) (39%) and MALT lymphomas (21%) prevailed. Nodal NHL with extranodal involvement (52,2%) were the most frequent. Nodal (21,8%) and extranodal (26%) lymphomas were less frequent. The most frequent target organs in lymphoproliferative disease (LPD) were lymph nodes (74%), salivary glands (45,5%), lungs (26%), bone marrow (19,5%), liver (17,5%), spleen (13%), lachrymal glands (6,5%). Waldeyer's throat ring (4%), oral cavity mucous membrane, ovary and brain (2%) were involved rarely. Immunoglobulin-secreting variant of lymphoma was revealed in 53,7% of cases. LPD developed predominantly in patients with systemic features and late stage of pSS. Significant increase of parotis, mixed monoclonal cryoglobulinemia, generalized lymphadenopathy, presence of more than 5 focuses of lymphoid infiltration in small salivary glands biopsies and thrombocytopenia were predictors of NHL development in pSS (p<0,001). Prolonged treatment with small doses of alkylating cytostatic agents (leukeran, cyclophosphan) decreased risk of LPD development in pSS (p><0,001). 5-year survival of patients from the moment LPD was diagnosed was 54% mainly due to high mortality in the group of patients with LCL. Survival of pSS patients with and without LPD s><0,001). Prolonged treatment with small doses of alkylating cytostatic agents (leukeran, cyclophosphan) decreased risk of LPD development in pSS (p<0,001). 5-year survival of patients from the moment LPD was diagnosed was 54% mainly due to high mortality in the group of patients with LCL. Survival of pSS patients with and without LPD significantly differed (p<0,001): after 20-year follow up survival was 32% in group with LPD and 76% in group without LPD. Probability of NHL development after 40-year course of pSS was 40% what allows to consider this disease simultaneously autoimmune and lymphoproliferative.

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