Responses of lymphocytes to Epstein-Barr virus in patients with primary immunodeficiencies

Several reports have demonstrated that the responses of B-cells to Epstein-Barr virus (EBV) are variable in common variable immunodeficiency (CVID). In this study in patients with selected primary immunodeficiencies, i.e., Bloom's syndrome, Wiskott-Aldrich syndrome or IgA deficiency, the responses of peripheral blood mononuclear cells (PBMCs) to EBV were investigated. In the two patients with Bloom's syndrome, PBMCs stimulated with EBV showed decreased proliferation and immunoglobulin production, suggesting a mild abnormality of B-cells. In patients with Wiskott-Aldrich syndrome, the responses were variable. In the patient with IgA deficiency, PBMCs responded normally to EBV in proliferation, whereas PBMCs responded poorly to EBV in IgA production, suggesting an abnormality only in the IgA production mechanism.