PRIMARY IMMUNODEFICIENCIES

Abstract

1. Two registries are to be established in association with WHO, with the object of gathering and recording information on cases of immunodeficiency from all over the world. One registry will comprise cases of primary immunodeficiency that have received transplants of any immunologically competent organ, cell, or cell product, e.g., bone marrow, thymus, or transfer factor. It will be organized by W. Hitzig and M. Seligmann. The other will comprise cases of malignant tumour found in patients with primary immunodeficiency; it will be organized by R. A. Good, who will also arrange for pathological material to be submitted for study by selected pathologists. The organizers will collate and periodically report on the material in both registries. 2. Standardization of diagnostic procedures and additional studies related to diagnostic tests are required. a. There is a need for standard preparations of antigens for assessing antibody formation as described. A sufficiently large batch of such antigens should be prepared, and aliquots should be sent, on request, for use by clinicians investigating immunodeficiency. b. Methods for measuring serum antibodies need to be standardized, and in vitro tests for the measurement of cell-mediated immunity, such as tests for cytotoxicity and macrophage migration inhibition factors, need to be further developed. 3. Therapeutic procedures should be standardized and assessed. a. Aggregates in immunoglobulin preparations for intramuscular or intravenous injection should be looked for with tests such as those recommended. b. National agencies responsible for immunoglobulin production should develop stable aggregate-free intact IgG, without fragments and with relatively normal in vivo survival, for the treatment of patients with immunodeficiency. c. It would be of value to determine the relative effectiveness of therapy with immunoglobulin and with plasma. Clinical trials might be designed for this purpose. d. Immunoglobulin preparations having high contents of specific antibodies would, if available, be valuable for treatment of patients with specific infections. e. Further research is required to develop new methods of destroying the biological activity of lymphocytes in whole blood. f. Pools of immunoglobulin, prepared as Cohn FII from donors with absence of One genetic allotype, may be valuable for the treatment of patients with immunodeficiency who have anti-allotype antibodies.