Severe combined immunodeficiency disease. Characterization of the disease and results of transplantation

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Abstract
Pretransplant and posttransplant data for 69 patients with severe combined immunodeficiency disease are presented. Both B [bone marrow-derived] and T [thymus-derived] lymphocyte functions were absent in approximately 80% of the children and markedly depressed in the remainder. Transplantation of marrow from HLA genotypically identical donors provided the highest 6-mo. survival rate (63%); 6-mo. survival rates for patients who received fetal tissue transplants (43%) or marrow from mixed leukocyte culture (MLC) negative donors (38%) were significantly higher (P < .05) than for patients treated with marrow from MLC positive donors (5%). Additional factors appeared to influence survival and the severity of graft-vs.-host (GVH) disease. Patients more than 6 mo. of age had more intense GVH disease than younger patients. Survival rates were lower and GVH disease more intense when boys received transplants from girl donors than the reverse.

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