Heterogeneity of “Acquired” or Common Variable Agammaglobulinemia

Raif S. Geha(American University of Beirut Medical Center), Eveline E. Schneeberger(American University of Beirut Medical Center), Ezio Merler(American University of Beirut Medical Center), Fred S. Rosen(American University of Beirut Medical Center)
New England Journal of Medicine
July 4, 1974
Cited by 203

Abstract

Abstract Circulating T (thymus-derived) and B (bone-marrow-derived) lymphocytes were assessed in 19 patients with common variable or acquired agamma-globulinemia. T-cell number and function were concomitantly depressed in five of these patients who had other debilitating illnesses. B-cell number was normal in 10 patients, increased in five, and markedly reduced in four. Transformation of B cells into immunoglobulin (Ig) secretory cells in the presence of a soluble T-cell-derived lymphocyte mitogenic factor (LMF) was studied in the 15 patients with B cells. B cells from nine of these patients, when stimulated with LMF, failed to synthesize Ig. In five other patients, B cells divided and made Ig, but they failed to secrete it. A single patient had a factor in his serum that inhibited activation of both normal and autologous B cells by LMF. Acquired or common variable agammaglobulinemia is a heterogeneous disease caused by defects occurring at various steps in the maturation pathway of the B cell into an...


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