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Abstract
Although infrequent, the development of non-Hodgkin's lymphoma (NHL) during the course
of Sjögren's syndrome represents a major complication. Nethertheless, most NHL developing
in patients with Sjögren's syndrome are B cell lymphomas involving the marginal zone,
localized to extranodal sites. Predictive factors include lymphadenopathy, splenomegaly,
neutropenia, cryoglobulinemia and low C4 levels. The treatment of B cell lymphomas
depends on its nature and localization. Biotherapies targeting the B lymphocyte, such
as rituximab, a chimeric monoclonal anti-CD20 antibody, or more recently targeting
BAFF can be proposed.