CLINICOPATHOLOGICAL FACTORS RELATING MALIGNANT-LYMPHOMA WITH SJOGRENS-SYNDROME

This study was undertaken to summarize the current status of the clini copathologic factors related to non-Hodgkin's lymphoma (NHL) developme nt in patients with Sjogren's syndrome (SS). Through a MEDLINE search, pertinent articles on SS, monoclonality, and NHL were found and revie wed. Malignant lymphoma description was based on the International Lym phoma Study Group classification. Patients with SS are known to have a n increased risk for developing B-cell NHL (B-NHL). However, such a co mplication occurs in less than 10% of patients, being reported mainly in those with primary SS. Extranodal low-grade B-NHL are observed most frequently. Persistent enlargement of parotid glands, adenopathy, mon oclonal gammopathy, and crossreactive idiotypes are all signs suggesti ng possible lymphoma evolution. Although monoclonality does not mean m alignancy unequivocally, it is considered to be a precursor for NHL de velopment in SS. Factors implicated into lymphomagenesis in SS include dysregulation in the mechanisms leading to apoptosis, hyperstimulatio n of B-1 cells, and an infectious agent. Polyclonal lymphoproliferatio n characterizing SS might in some instances transform into monoclonal, and then to malignancy. Further studies on the mechanism whereby NHL develops in SS are warranted. (C) 1996 by W.B. Saunders Campany