Normal Serum Immunoglobulin Levels Research Articles

IMMUNOLOGICAL INCOMPETENCE IN CARTILAGE-HAIR HYPOPLASIA

We have studied the immunological capacity of 23 patients suffering from short-limbed growth failure due to metaphyseal chondrodysplasia associated with normal skull and vertebral column. Most of the patients had hypoplastic hair, but this feature was variable even between affected siblings. Many had recurrent or prolonged infections. Humoral immunity was unaffected as indicated by normal serum immunoglobulin levels, normal number of immunoglobulin containing cells in the intestinal mucosa, normal secretion of immunoglobulin on mucosal surfaces and normal production of antibodies against viruses. Skin reactivity to tuberculin was depressed. The blast transformation of lymphocytes on stimulation with phytohaemagglutinin was below 95% confidence limit of controls in all but one patient. This patient had normal hair. The in vitro response to tuberculin was also absent or siight. However, the percentage of lymphocytes forming non-immune rosettes with sheep red cells was only slightly decreased. This test is considered to quantify the T-lymphocytes. The discrepancy between these findings can be explained by functional deficiency of T-lymphocytes. Otherwise, the tests may measure dif. functions of ”.

Evaluation of T Lymphocyte Effector Function in Immunodeficiency Diseases: Abnormality in Mitogen-Stimulated Interferon in Patients with Selective IgA Deficiency

Abstract Interferon production in response to the mitogens phytohemagglutinin and pokeweed was studied as a new index of the competence of thymus-derived (T) lymphocyte effector function in patients with various immunodeficiency states. The results indicated that three of four patients with selective IgA deficiency and normal T cell proliferative response had a depressed T cell interferon response. Two patients who had received successful thymus transplants had a normal T cell interferon response. Before transplantation one had been diagnosed as severe combined immunodeficiency, and the other, thymic hypoplasia with normal serum immunoglobulin levels. By contrast, a patient with thymic hypoplasia and absent serum IgA, low IgM and normal IgG levels, who had had an unsuccessful thymic graft, had a markedly depressed interferon response. Normal T cell interferon responses were seen in one patient with congenital sex-linked hypogammaglobulinemia and three patients with acquired hypogammaglobulinemia.