CD21-/low B cells: a snapshot of a unique B cell subset in health and disease

B cells represent one of the cellular components of the immune system thatprotects the individual from invading pathogens. In response to the invader,these cells differentiate into plasma cells and produce large amounts of antibodiesthat bind to and eliminate the pathogen. A hallmark of autoimmune diseases isthe production of autoantibodies i.e. antibodies that recognize self. Those that areconsidered pathogenic can damage tissues and organs, either by direct binding orwhen deposited as immune complexes. For decades, B cells have been consideredto play a major role in autoimmune diseases by antibody production. However, aspathogenic autoantibodies appear to derive mainly from T cell dependentresponses, T cells have been the focus for many years. The successful treatment ofpatients with autoimmune diseases with either B cell depletion therapy(rituximab) or inhibition of B cell survival (belimumab), suggested that notonly the autoantibodies but also other B cell features are important. This hascaused a surg e of interest in B cells and their biology resulting in theidentification of various subsets e.g. regulatory B cells, several memory B cellsubsets etc. Also, in other conditions such as chronic viral infect ions and primaryimmunodeficiency, several B cell subsets with unique characteristics have beenidentified. In this review, we will discuss one of these subsets, a subset that isexpanded in conditions characterized by chronic immune stimulation. This B cellsubset lacks, or expresses low, surface levels of the complement receptor 2(CD21) and has therefore been termed CD21-/lowB cell