Histiocytosis-X.
Twelve of 17 patients with histiocytosis-X were immunologically abnormal, as shown by the presence of circulating lymphocytes spontaneously cytotoxic to cultured human fibroblasts or of antibody to autologous erythrocytes. The patients also had a notable lack of histamine H2 surface receptors on their T lymphocytes, suggesting a suppressor-cell deficiency. The lymphocyte abnormalities were reversed in vitro after incubation in a crude extract of calf thymus gland, and therefore all 17 patients were treated with daily intramuscular injections of this extract. With this therapy, 10 patients entered complete remission -- a response at least as good as that observed in historical controls treated with chemotherapy. A positive clinical response was associated with an increase in the number of T-cell histamine H2 receptors to normal levels and with correction of the other immunologic abnormalities. The results of this preliminary study justify a larger prospective clinical trial of thymic extract and further investigation of the immunoregulatory mechanisms in histiocytosis-X.
D002462:Cell Membrane / Q000276:immunology; D002675:Child, Preschool; D002986:Clinical Trials as Topic; D003601:Cytotoxicity Tests, Immunologic; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D011968:Receptors, Histamine / Q000032:analysis*; D011970:Receptors, Histamine H2 / Q000032:analysis*; D012075:Remission, Spontaneous; D012397:Rosette Formation; D012882:Skin Tests; D013601:T-Lymphocytes / Q000276:immunology*; D050378:T-Lymphocytes, Regulatory / Q000276:immunology; D013949:Thymus Extracts / Q000627:therapeutic use*
D011968:Receptors, Histamine; D011970:Receptors, Histamine H2; D013949:Thymus Extracts