Prognosis and Outcome of Non-Hodgkin Lymphoma in Primary Sjögren Syndrome

Abstract

Sjögren syndrome (SS) has been associated with the development of non-Hodgkin lymphoma (NHL). From a cohort of 584 SS patients followed in our department from 1980 to 2010, we retrospectively analyzed 53 consecutive NHL cases. Considerations included histologic type, clinical manifestation and NHL staging, treatment, response rate and overall survival (OS), event-free survival (EFS), and standardized mortality ratio (SMR).Mucosa-associated lymphoid tissue (MALT) lymphomas constituted the majority (59%) of NHL subtypes, followed by nodal marginal zone lymphomas (NMZLs) (15%) and diffuse large B-cell lymphomas (DLBCLs) (15%). Six lymphoma patients died during the median follow-up of 40.8 months. The corresponding age/sex-adjusted SMR of SS with and without NHLs versus the general population was 3.25 (95% confidence interval [CI] 1.32-6.76) and 1.08 (95% CI, 0.79-1.45), respectively. A "watch and wait" policy was adopted for 9 patients with asymptomatic localized salivary MALT lymphomas. Eight patients with limited-stage MALT lymphomas and extraglandular manifestations were treated with rituximab. Ten MALT lymphoma patients with disseminated disease received chemotherapy with or without rituximab. The 3-year OS and EFS in patients with MALT lymphomas was 97% and 78%, respectively. Rituximab plus CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) was the chosen therapeutic intervention for patients with DLBCLs. A successful outcome was recorded for this group, with 100% OS and EFS at 3 years. Patients with NMZLs had a less favorable outcome, with a 3-year OS of 80% and EFS of 53%. Our results describe the course and prognosis of SS-associated NHL and highlight the need for a risk-stratified treatment approach.

Lymphome bei rheumatischen Erkrankungen

“…Eine retrospektive Studie an 53 Patienten zeigt ein 3-Jahres-Überleben von 97 % sowie ein ereignisfreies Überleben (EFS) von 78 % [61]. …”

Renal Lymphoma: Unusual Lymphoproliferative Manifestation of Sjögren’s Syndrome

“…The relationship between SS and lymphoma has been known since the 1960s 3 , with recent metaanalyses supporting a relative risk of lymphoma development ranging from 5.4 to 16 1,4,5,6 . The majority of lymphomas developing in patients with SS are low-grade marginal zone B-cell lymphomas, particularly of the MALT variety 7 , with major sites including the salivary glands, buccal mucosa, lacrimal glands, lung, stomach, and cervical lymph nodes 2 .…”

“…The majority of lymphomas developing in patients with SS are low-grade marginal zone B-cell lymphomas, particularly of the MALT variety 7 , with major sites including the salivary glands, buccal mucosa, lacrimal glands, lung, stomach, and cervical lymph nodes 2 . But lymphomas in SS can also progress toward a less differentiated cell type, with DLBCL accounting for 15% of NHL cases in SS 1,4,6 .…”

Oral manifestations of systemic disease

“…The tumours are usually low grade marginal zone lymphoma (MALT), but can also be follicle centre, diffuse B cell lymphoma (DBCL) or lymphoplasmacytoid lymphoma. 62,63 OTHER ORAL MANIFESTATIONS The present article has focused upon the impact of systemic disease on the oral mucosa and salivary glands but a very wide range of other symptoms and/or signs of systemic disease can arise in the mouth or adjacent face as a consequence of disorders of non-oral structures (summarised briefly in Table 5). …”