In vivo and in vitro effects of thymosin and adenosine deaminase on adenosine-deaminase-deficient lymphocytes.
Two siblings with adenosine deaminase deficiency were studied before and during "enzyme replacement" therapy (partial exchange transfusions with normal red cells containing the missing enzyme). The younger sib showed improvement of immunologic function during red-cell therapy alone, whereas in the older sib this improvement occurred only when the transfusions were supplemented by thymosin injections. Their clinical courses correlated with in vitro findings: lymphocytes from the younger sib differentiated to T-cell-rosette-forming cells upon addition of adenosine deaminase alone; lymphocytes from the older sibling required supplemental thymosin to form these cells. Thymic factors appear to influence the response to transfusion therapy in some patients deficient in adenosine deaminase, and supplementation of red-cell transfusion with thymic factors may be required.
D000243:Adenosine Deaminase / Q000008:administration & dosage / Q000097:blood / Q000172:deficiency* / Q000627:therapeutic use*; D004912:Erythrocytes / Q000201:enzymology; D005078:Exchange Transfusion, Whole Blood; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000188:drug therapy / Q000209:etiology / Q000235:genetics / Q000628:therapy*; D007223:Infant; D008214:Lymphocytes / Q000201:enzymology*; D008297:Male; D009700:Nucleoside Deaminases / Q000172:deficiency* / Q000627:therapeutic use*; D012397:Rosette Formation; D013601:T-Lymphocytes / Q000276:immunology; D013947:Thymosin / Q000008:administration & dosage / Q000627:therapeutic use*; D013951:Thymus Hormones / Q000627:therapeutic use*
D013951:Thymus Hormones; D013947:Thymosin; D009700:Nucleoside Deaminases; D000243:Adenosine Deaminase