Abstract

Serial immunologic measurements were used to study the metabolic behavior of the immune globulins (γG-, γM- and γA-globulins) in patients with agammaglobulinemia after plasmapheresis and plasma infusion and in newborn infants after exchange transfusion. These studies were supplemented by metabolic and distribution studies of I131-labeled γA-globulin (isolated from serum or breast milk) and I131-labeled γG-globulin in normal and agammaglobulinemic subjects. The therapeutic benefit of periodic plasma infusions in patients with agammaglobulinemia and the Aldrich syndrome was also assessed.

In the agammaglobulinemic patients, the mean half-lives of γG-, γM- and γA-globulin were 32, 9.6 and 5.9 days, respectively. In the transfused infants, the mean half-lives of γM- and γA-globulins were 7.4 and 4.3 days, respectively. Agreement existed between simultaneously determined immunologic and radioactive survival times, except when I131-labeled γA-globulin isolated from serum was used; this preparation had a shorter half-life than the γA-globulin of infused plasma, probably as a result of denaturation during the isolation procedure. Studies on 2 normal and 3 agammaglobulinemic subjects showed that 65 to 85 per cent of breast milk I131-labeled γA-globulin was distributed within the tissues. I131-labeled γA-globulin was not demonstrable in the breast milk of 2 lactating women or in the saliva of 2 normal subjects. No γA-globulin could be demonstrated in the saliva of an agammaglobulinemic patient after plasma infusion which raised the serum γA-globulin concentration to 50 mg./100 ml.

The use of plasma instead of commercial γ-globulin for the therapy of immunologic deficiency states has several advantages. Plasma contains all three immune globulins, provides greater quantities of γG-globulin than can be given by intramuscular injections, and is more acceptable to the patient. Because of the risk of serum hepatitis, this mode of therapy in the routine management of agammaglobulinemia is endorsed only if special precautions are taken. A therapeutic trial of plasma infusions in 2 patients with the Aldrich syndrome gave promising results.

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