Histiocytosis-X

N Engl J Med. 1981 Jan 15;304(3):146-53. doi: 10.1056/NEJM198101153040304.

Abstract

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.

Publication types

  • Clinical Trial
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Cell Membrane / immunology
  • Child, Preschool
  • Clinical Trials as Topic
  • Cytotoxicity Tests, Immunologic
  • Female
  • Histiocytosis, Langerhans-Cell / immunology*
  • Histiocytosis, Langerhans-Cell / therapy
  • Humans
  • Infant
  • Male
  • Receptors, Histamine / analysis*
  • Receptors, Histamine H2 / analysis*
  • Remission, Spontaneous
  • Rosette Formation
  • Skin Tests
  • T-Lymphocytes / immunology*
  • T-Lymphocytes, Regulatory / immunology
  • Thymus Extracts / therapeutic use*

Substances

  • Receptors, Histamine
  • Receptors, Histamine H2
  • Thymus Extracts