Abstract
To test the hypothesis that histiocytosis-X may be a primary autoimmune disorder and not a true cytoproliferative malignancy, we assayed the reactivity to autologous antigens of Ficoll-Hypaque separated peripheral blood mononuclear cells (PBM) from controls and patients with histiocytosis-X. The PBM of 4/4 patients demonstrated reactivity to autologous antigens in one or more assays. None of 10 controls demonstrated any autoreactivity in the assays. The assays used were: cytotoxicity of 14C labelled cultured autologous fibroblasts, cytotoxicity of 51Cr labelled autologous lymphocytes, and stimulation of PBM protein synthesis by actinomycin-D treated autologous lymphocytes. Pre-incubation of patients' PBM with thymic humoral factor (THF) eliminated the abnormal autoreactivity in all three assays. Because THF abolished in vitro reactivity, one patient, a chemotherapy failure, was treated with THF. He is now in complete remission, requires no additional THF therapy, and his PBM no longer demonstrates autoreactivity. Histiocytosis-X may be caused by inadequate regulation of autoreactive lymphocytes, whose response to autologous antigens produces the manifestations of the disease. The lack of regulation may be due to a thymic deficiency that can be corrected with THF replacement therapy.
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Osband, M., Parkman, R. 642 DEMONSTRATION THAT HISTIOCYTOSIS-X MAY BE AN AUTO-IMMUNE DISORDER AND SUCCESSFUL TREATMENT WITH THYMIC HUMORAL FACTOR. Pediatr Res 12 (Suppl 4), 470 (1978). https://doi.org/10.1203/00006450-197804001-00647
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DOI: https://doi.org/10.1203/00006450-197804001-00647