Predicting the Outcome of Sjogren’s Syndrome-Associated Non-Hodgkin’s Lymphoma Patients

Abstract

BackgroundNon-Hodgkin's lymphoma (NHL) development in Sjögren’s syndrome (SS) remains a potentially lethal complication and efforts should focus on the identification of predictors that could aid in appropriate therapeutic decisions.MethodsIn order to identify potential prognostic factors for outcome in SS-associated NHL, we retrospectively analyzed a cohort of 77 patients, diagnosed with NHL according to WHO classification criteria and meeting the American-European Consensus Classification (AECC) criteria for SS and examined the effect of SS-activity (defined as the EULAR SS disease activity index-ESSDAI) in the prognosis of SS-related NHLs, as defined in terms of overall and event-free survivals (OS and EFS). An event was defined as lymphoma relapse, treatment failure, disease progression, histological transformation or death. The effect of NHL clinical and laboratory characteristics was also investigated.ResultsMALT lymphomas constituted the majority (66.2%) of lymphomas. During the follow-up (median = 57.93 months), the 5-year OS was 90.91% (95% CI: 82.14–95.80%) and the EFS was 77.92% (95% CI: 67.37–85.82%). Patients with high ESSDAI score at lymphoma diagnosis had a greater risk for death (OR = 5.241, 95% CI: 1.034–26.568) or for event (OR = 4.317, 95% CI: 1.146–9.699, p = 0.008). These patients had also significantly worse EFS (HR = 4.541, 95% CI: 1.772–11.637) and OS (HR = 5.946, 95% CI: 1.259–28.077). In addition, post-chemotherapy ESSDAI improvement was significantly lower in patients who had experienced an event (p = 0.005). An unfavorable International prognostic index (IPI) score (high-intermediate/high) was associated with high risk of death and event (OR = 13.867, 95% CI: 2.656–72.387 and OR = 12.589, 95% CI: 3.911–40.526, respectively), worse EFS (log-rank p<0.001, HR = 8.718, 95% CI: 3.477–21.858), as well as with worse OS (log-rank p<0.001, HR = 11.414, 95% CI: 2.414–53.974). After adjustment for identified risk factors, IPI score retained a significant prognostic role following by a strong effect of ESSDAI in survival outcomes.ConclusionsAt the point of NHL diagnosis, IPI and ESSDAI might be proved useful predictive tools in SS-associated lymphoma prognosis, directing to a more patient-tailored approach.

Pulmonary MALT Lymphoma in Patients with Sjögren’s Syndrome

“…MALT lymphomas in patients with pSS are most frequently located in the parotid gland, although localization in other sites such as thyroid, stomach and lung has also been described. [2][3][4] Extranodal MZL of the lung, sometimes referred to as bronchial-associated lymphoid tissue (BALT) lymphoma, is a rare pulmonary lymphoid malignancy. 5,6 Occasionally, a preexisting autoimmune disorder, most notably pSS, is observed in patients with pulmonary MALT lymphomas.…”

Clinical features and outcome of lymphoma patients with pre‐existing autoimmune diseases

“…MALT lymphoma constitutes the majority of lymphomas in SS patients. 21 . By promoting the hyperactivity and lymphoproliferation of B cells, B-lymphocyte stimulator 22 and FMS-like tyrosine kinase 3 ligand 23 are considered to be the essential factors for MALT lymphoma development in SS patients.…”

Lymphoproliferative Disorders Associated with Sjögren Syndrome

“…The 5-year EFS was 86.27 % for patients with MALT, 62.5 % for patients with NMZ lymphoma, 50.0 % in DLBC lymphoma patients, and 83.3 % for other lymphoma subtypes. Survival differences between MALT and DLBC lymphomas were statistically signifi cant [ 50 ]. The studies also revealed that the severity of SS disease (using the disease activity score) negatively impacts on the prognosis of NHL patients.…”

“…Of the 77 patients diagnosed with NHL, MALT lymphoma constituted the majority (51/77, 66.2 %) of NHL subtypes, followed DLBC (12/77, 15.6 %) and nodal marginal zone (NMZ) lymphomas (8/77, 10.4 %). Compared to patients with DLBC, MALT lymphoma patients were signifi cantly younger (median age was 55 versus 69 years, respectively) and developed lymphoma much earlier (median time from SS diagnosis to MALT development was 65.80 versus 97.54 months, respectively) [ 50 ].…”

“…The authors also recognized additional predictors of lymphoma prognosis, such as an elevated the lymphoma international prognostic index (IPI) score and bone marrow involvement. After adjustment for identifi ed risk factors, IPI score retained a signifi cant effect on survival outcomes followed by a strong trend for SS disease activity score [ 50 ].…”