Abstract
General consideration of primary immunodeficiency diseases (PID) was discussed on the classification of the diseases presented by WHO scientific group. PID were categorized according to the presumed level of the cellular defect.
Thymus had the important role in the ontogeny of T cells. T precursor cells were differentiated under the influence of thymus hormones.
Four different kind of putative thymus hormones were presented briefly. Using the rosette inhibition assay, serum activity of facteur thymique sérique (FTS) was determined for various PID. Patients with DiGeorge syndrome always had low FTS levels. Twenty one out of 23 patients with severe combined immunodeficiency showed low levels but only two had FTS activity within the normal range. Determination of FTS activity might provide a new parameter in the aspect of thymus function in vivo.
Cellular engineering was the specific treatment for PID. Bone marrow transplantation (BMT) was the best method and it could cure many PID permanently as far as a matched donor was present. Effort must be done to find a good method for allogeneic BMT from an unrelated donor or to search the alternative way, for example using fetal organs.
Immunological substitution was also mentioned.
