Common variable immune deficiency (CVID) presenting as an autoimmune disease: role of memory B cells

Common variable immunodeficiency (CVID) is a clinically heterogeneous disorder. Most often patients present with recurrent sinopulmonary infections, although it may present with autoimmune manifestations. Immune cytopenias, particularly thrombocytopenia and hemolytic anemia, are the most commonly observed. While the pathophysiology of CVID remains elusive, in many patients it may be due to an intrinsic B cell defect. Memory B cells (CD27+) in particular, have been noted to correlate with certain aspects of the disease. High numbers of IgM+ memory B cells appear to correlate with the presence of infections, whereas decreased numbers of switched memory B cells correlate with lower serum IgG levels and increased rates of autoimmune features. Because of these defects in the memory B cell compartment, there is a greater potential risk for infection and related complications. Review of the literature suggests that splenectomy should be avoided in patients with immune cytopenia and CVID and that serum immunoglobulins should be obtained in patients presenting with immune cytopenias to screen for CVID.