A 55-year-old male with a history of recurrent bacterial and viral infection was found to have hypogammaglobulinaemia in association with a benign thymoma. The patient lacked eosinophils and was deficient in basophils in both the peripheral blood and bone marrow. The absence of eosinophils in a skin challenge known to recruit these cells, and below normal total peripheral blood leucocyte histamine content further suggests a deficiency of eosinophils and basophils respectively. Abnormal suppressor T cell function was documented both phenotypically, using OKT monoclonal antisera, and functionally in pokeweed mitogen stimulated cultures. This patient is the first reported with a deficiency of both eosinophils and basophils occurring in the presence of hypogammaglobulinaemia and thymoma; these abnormalities may be related to the abnormal suppressor T cell function that is present.
A quantitative technique has been developed for measurement of immunoglobulin production by human rectal mucosa in vitro. The technique overcomes the problem of serum proteins trapped in the tissue by parallel measurements of Ig and human serum albumin (HSA) over a 6 day period. IgG, IgA, IgM and HSA were measured in supernatant fluids using sensitive radioimmunoassays. The technique has demonstrated IgG and IgA synthesis by rectal mucosa in vitro. The conclusion that the observed IgA and IgG was synthesized in vitro was supported by the demonstration that the production could be increased by pokeweed mitogen and blocked by emetine. This culture system has been applied to measure Ig synthesis by the rectal mucosa of immunodeficient patients.
mitogen and IgM measured seven days later in g±g/ml. To assess suppressor cells equal numbers (6 x 10') of normal T cells or T cells from a patient with thymoma were added. The data show that the patient's T cells, whether treated (Tmito) or untreated (T) with mitomycin C, suppressed immunoglobulin production while normal T cells caused an increase. This experiment was repeated with similar results (de Gast et al., 1979a).
Improved methods for separating B and T cells have made it possible to assess the functions of these cells. These techniques have been used to study the T dependence of B-cell immunoglobulin production in response to pokeweed mitogen. In the present study they have been used to reinvestigate the pathogenesis of "variable" primary hypogammaglobuliaemia. Twelve patients were studied by separating their B and T cells and culturing them with normal cells. The results show that none of the patients have normal B cells. In cultures containing high ratios of T cells to B cells we have found a marked reduction in B-cell immunoglobulin production both with T cells from normal donors and from patients. T cells from all but one of the patients helped immunoglobulin production and reduced T helper activity was only seen in patients whose B cells were very abnormal. The results suggest that a B-cell defect is the cause of the low serum immunoglobulin in all the patients we have studied.
Five patients with primary hypogammaglobulinaemia developed a severe polyarthritis that had some features in common with rheumatoid arthritis. Their joint disease could be distinguished from rheumatoid arthritis, however, by the dramatic improvement after gammaglobulin treatment. The arthritis of hypogammaglobulinaemia can, therefore, be included among the few potentially curable polyarthritides.
Grandfather 2 2 uncles 1of familial pattem in the understanding of this disease.A positive family history of peptic ulcer is an important feature and is one of the basic characteristics of a duodenal ulcer in children. SummaryThe families of 37 children with the established diagnosis of duodenal ulcer were studied to assess the incidence of peptic ulcer in the relatives of these children. 23children had a positive family history of peptic ulcer: 19 children had one or other parent or sib affected with the disease.Positive family history is an important characteristic of duodenal ulcer in children.