Auto-immunosuppression: Recurrent infections associated with immunologic unresponsiveness in the presence of an auto-antibody to IgG

Comparative Study of Bone Marrow and Blood B Cells in Infantile and Acquired Agammaglobulinemia

“…Several patients with selective IgA deficiency were found to have in their serum anti-IgMI of the IgG class (22,23). Recently a high titer anti-IgG of the IgMI class was detected in a patient with AGG with recurrent infections (24). In a recent study anti-IgM antibodies were detected in 23.8% of 18 patients with various types of immunodeficiencies (25).…”

“…This could be attributed to the heterogeneity of this group of patients and/or to the severity of their disease. More work is needed to look for the variables in this disease and for possible B cell defects at various levels of maturation following their emigration from the central lymphoid compartment. This study and several other recent publications (6,12,17,24) will help in the understanding of clinical disease states associated with abnormal B cell function. Defects at one or more of the maturation steps of the stem cell to mature B cells have to be considered in antibody deficiency states.…”

Autoantibodies to B Lymphocytes in a Patient with Hypoimmunoglobulinemia

“…Lymphocytotoxins have been recently reported in a variety of human diseases, including systemic lupus erythematosus and inflammatory bowel diseases (2,3). The data on the occurrence of such anti-lymphocyte antibodies in patients with ID are very scarce (4,5). In the hitherto reported cases neither a definite anti-B (bone marrow-derived) or anti-T (thymus-derived) specificity nor a clear relationship with immunodepression have been established.…”

“…Whether this negative finding is related to the neoplastic nature of the CLL cells or to the various levels in the differentiation pathway of the B-cell line where proliferating clones from individual CLL patients appear to be blocked (6) (4). In another boy with recurrent infections and deficiency of both cellular and humoral immunity, a high titer IgM antibody to IgG was found and was thought to be responsible for a non-HLA associated lymphocytotoxicity which was blocked by IgG (5). In patient D.S., it may be assumed that the antibody to B lymphocytes is an autoantibody and that it played a crucial role in the pathogenesis of hypoimmunoglobulinemia, since the withdrawal of this antibody by plasmapheresis was followed by a dramatic and transitory increase in the number of circulating B lymphocytes.…”