Hyperimmunoglobulinaemia associated with absent suppressor cells, aplastic anaemia and trisomy 8.
A child with aplastic anaemia and trisomy 8 in bone marrow cells was observed to have extremely high levels of circulating immunglobulins. Concanavalin A (con A)-inducible suppressor cells were assessed in the patient's peripheral blood by their ability to suppress both the proliferative response of normal allogeneic cells activated in a two-way mixed lymphocyte culture with and without added phytohaemagglutinin (PHA) and the ability of normal allogeneic cells to produce the lymphokine leucocyte inhibitory factor when activated by PHA. Con A consistently failed to induce suppressor cells from the patient but not from control lymphocytes. It is suggested that the lack of suppressor cells may account for uncontrolled B-cell proliferation and associated hypergammaglobulinaemia.
D000741:Anemia, Aplastic / Q000150:complications*; D001856:Bone Marrow Examination; D002675:Child, Preschool; D002906:Chromosomes, Human, 6-12 and X; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000150:complications* / Q000276:immunology; D007070:Immunoglobulin A / Q000276:immunology; D007074:Immunoglobulin G / Q000276:immunology; D007075:Immunoglobulin M / Q000276:immunology; D007621:Karyotyping; D050378:T-Lymphocytes, Regulatory / Q000502:physiology; D014314:Trisomy; D014960:X Chromosome
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M