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EXTRANEOUS LYMPHOCYTIC HL‐A ANTIGENS IN SEVERE COMBINED IMMUNODEFICIENCY DISEASE

Lymphocytes of three patients with severe combined immunodeficiency disease were markedly anomalous in having extraneous HL-A specificities. Data from family studies disclosed that the phenotypic specificities expressed were in excess of the predicted genotypic antigens. Contrary to the current theories of HL-A, which limit the number of antigens to four, as many as 12 specificities were found in one patient. No stimulation by the extraneous antigens was revealed by the mixed leukocyte culture (MLC) test with a sibling of this patient who was presumed to be genotypically HL-A-identical. Bone marrow transplantation from this genotypically HL-A-identical sibling resulted in heightening immunoglobulin levels and successful clinical outcome to the present time (11 months). Although the phenotyping anomaly disappeared 2 weeks after transplantation, it subsequently reappeared and has persisted for 5 months. A possible association of immunoglobulins and histocompatibility antigens with respect to synthesis and membrane sites is discussed.