Preliminary Classification of Nonmalignant B Cell Proliferation in Sjögrenʹs Syndrome: Perspectives on Pathobiology and Treatment Based on an Integrated Clinico-Pathologic and Molecular Study Approach

A classification of nonmalignant lymphoproliferation in Sjögrenʹs syndrome is presented, based on the results of international meetings regarding Sjögrenʹs syndrome-associated lymphomagenesis and on our results of a clinico-pathologic and molecular study and long-term follow-up in well-characterized patients. Sjögrenʹs syndrome pathobiology has similarities to hepatitis C virus-related B-cell lymphoproliferation. Antigen stimulation with the preferential expansion of rheumatoid factor-positive clones and specific immunoglobulin gene expression and recombination represent key biologic events in lymphoproliferation. This classification is based on the couplingof molecular and histological studies and may result in more selective treatment approaches.