Human immunodeficiency resulting from a maturational arrest of germinal center B cells.
Common variable immunodeficiency (CVI) is an acquired human disorder involving a striking and heterogeneous maturational defect of B lymphocytes. In this study, we used a recently developed VH gene utilization assay to analyze the abundance of developmentally restricted and unrestricted V genes in blood B cells from nine CVI patients. Unrestricted clones (bearing rearranged VH5, VH4, or VH6 genes) were present in normal abundance in this group of CVI patients. However, clones bearing VH3L, a subgroup of the VH3 family normally abundant in blood B cells but absent in B cells at the germinal center stage, were deficient in seven of nine CVI patients. Based on these findings and a reconsideration of previously reported B cell features in CVI, we propose that the disorder represents in most cases a maturational arrest of B cells at the germinal center stage.
Braun
J
J
Department of Pathology, UCLA School of Medicine 90024-1732.
Galbraith
L
L
Valles-Ayoub
Y
Y
Saxon
A
A
D000944:Antigens, Differentiation, B-Lymphocyte / Q000235:genetics; D016175:B-Lymphocyte Subsets / Q000276:immunology*; D001483:Base Sequence; D005803:Genes, Immunoglobulin; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D007135:Immunoglobulin Variable Region / Q000235:genetics*; D007153:Immunologic Deficiency Syndromes / Q000235:genetics* / Q000276:immunology; D008213:Lymphocyte Activation; D008969:Molecular Sequence Data; D016133:Polymerase Chain Reaction
D000944:Antigens, Differentiation, B-Lymphocyte; D007143:Immunoglobulin Heavy Chains; D007135:Immunoglobulin Variable Region