Accessory and T cell defects in acquired and inherited hypogammaglobulinaemia.
Cellular defects in patients with common variable hypogammaglobulinaemia (CVH) and X-linked agammaglobulinaemia (XLA) have been studied in vitro, using a mitogen-driven system of immunoglobulin production. We have confirmed our previous finding of impaired low-density (dendritic) accessory cell function in CVH and now show that accessory cell function is normal in XLA. We demonstrate that macrophage accessory function is normal in CVH. T cell help for IgM production is also deficient in CVH, and T cell help in XLA is also abnormal for both IgG and IgM. Some XLA patients have excessive T suppressor activity. The contribution of these defects to the clinical states is discussed.
Rozynska
K E
KE
Division of Immunological Medicine, Clinical Research Centre, Harrow, England.
Spickett
G P
GP
Millrain
M
M
Edwards
A
A
Bryant
A
A
Webster
A D
AD
Farrant
J
J
D000361:Agammaglobulinemia / Q000235:genetics / Q000276:immunology*; D000938:Antigen-Presenting Cells / Q000502:physiology*; D003713:Dendritic Cells / Q000502:physiology; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D008264:Macrophages / Q000502:physiology; D008297:Male; D013601:T-Lymphocytes / Q000502:physiology*; D006377:T-Lymphocytes, Helper-Inducer / Q000502:physiology; D050378:T-Lymphocytes, Regulatory / Q000502:physiology; D014960:X Chromosome