X-linked agammaglobulinemia a clinical and molecular analysis

HD Ochs, CIE Smith - Medicine, 1996 - journals.lww.com
X-linked agammaglobulinemia (XLA), 1 of the first primary immunodeficiency disorders to be
identified (9), remains the prototypic example of a primary immunodeficiency disease in
which the defectis lim-ited to cells of the B-lymphocyte system and their function. XLA is
characterized by a profound deficiency of B lymphocytes due to an arrest in B lymphocyte
development, resulting in hypogammaglob-