To improve the understanding of diagnosis and treatment of patients with primary The data of clinical features, laboratory Sjögren's syndrome (pSS) complicated with lymphoma.
findings, therapeutic response and follow-up of patients with primary Sjögren's syndrome complicated with lymphoma from January 2006 to January 2011 in our single center were retrospectively analyzed.
Totally twelve inpatients with pSS complicated with lymphoma were diagnosed, which accounted for 1.29% of newly-diagnosed lymphoma inpatients during the same period. The characteristic immunologic changes were hyperimmunoglobulinemia, hypocomplementemia and decrease of CD4 T cell number. In our study, non-Hodgkin's lymphoma (NHL) was the most common type, and the main pathological subtype was diffuse large B cell lymphoma (DLBCL). Most of the patients were in advanced stages, Ann Arbor stage IIl-IV, at diagnosis. Extranodal involvement was common, most frequently in the livers and the lungs. All of the patients received combination chemotherapy. Most of the NHL patients received CHOP/R-CHOP-like regimens, and the Hodgkin's lymphoma (HL) patient received AVD regimen. The median follow-up time was 27 months (range 1-56 months). In terms of median survival time and overall survival there were no statistical significant differences between both low C3 and low C4 group and control group (P > 0.05). In terms of median survival time and overall survival there were no statistical significant differences between rituximab treatment group and control group (P > 0.05).
The patients with pSS complicated with lymphoma were not uncommon clinically. Hypocomplementemia could not be identified as a risk factor for the prognosis of pSS complicated with lymphoma in our study. Although expected prognosis of these patients was unfavorable, we found that treatment with rituximab combination chemotherapy could not improve the therapeutic effects and survival of patients with pSS complicated with lymphoma.