Current Concepts and Treatment of Histiocytosis X (Langerhans’ Cell Histiocytosis)

  • M. E. Nesbit
Part of the UICC International Union Against Cancer book series (1360)


Histiocytosis is a general term used to indicate certain pathological disorders of the reticuloendothelial system [1]. Cells of this system have been classified as the mononuclear phagocytic system (MPS) because of their common origin and function [2]. They constitute an important defence system in the body against infections and foreign particulate matter, as well as being an important cellular component of the afferent limb of the immune response. The cells probably all originate from the pro-monocyte in the bone marrow and give rise to two different cell lineages. One line includes the peripheral monocyte which, in turn, gives rise to the tissue macrophage (histiocyte), while the other line includes the cells referred to as the dentritic cell population. This latter cell line is intimately involved with the thymus-dependent regions of the body and important in antigen processing, and the cells are powerful stimulators of mixed leukocyte reactions [3].


Eosinophilic Granuloma Bony Lesion Afferent Limb Sinus Histiocytosis Birbeck Granule 
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  1. 1.
    Aschoff L (1924) Das reticulo-endotheliale System. Ergeb Inn Med Kinderheilkd 26: 1Google Scholar
  2. 2.
    Langevoort HL, Cohn ZA, Hirsh JG, Humphrey JH, Spector WJ, van Furth R (1970) The nomenclature of mononuclear phagocytic cells. In: van Furth R (ed) Mononuclear phagocytes. Davis, Philadelphia, pp 1–6Google Scholar
  3. 3.
    Steiman RM (1981) Dendritic cells. Transplantation 31: 151–155CrossRefGoogle Scholar
  4. 4.
    Dargeon H (1966) Reticuloendotheliosis in childhood. Thomas, Springfield, pp 5–127Google Scholar
  5. 5.
    Lichtenstein L (1953) Integration of eosinophilic granuloma of bone, “Letterer-Siwe Disease” and “Schüller-Christian Disease” as related manifestations of a single nosologic entity. Arch Pathol 56: 84–102Google Scholar
  6. 6.
    Blazar B, Dehner LP, Nesbit ME (1983) The histiocytic disorders. In: Pochedly C (ed) Cancer therapy in children. Slack, Thorofare, pp 128–144Google Scholar
  7. 7.
    Birbeck MS, Breathnach AJ, Everall JD (1951) An electron microscopic study of basal melanocytes and high-level clear cells ( Langerhans’) cells in vitiligo. J Invest Dermatol 37: 51–64Google Scholar
  8. 8.
    Rousseau-Merck MF, Barbey S, Jaubert F, Bach MA, Chatenoud L, Nezelof C (1983) Reactivity of histiocytosis X cells with monoclonal antibodies. Pathol Res Pract 177: 8–12PubMedCrossRefGoogle Scholar
  9. 9.
    Thomas JA, Janossy G, Chilosi M, Pritchard J, Pincott JR (1982) Combined immunological and histochemical analysis of skin and lymph node lesions in histiocytosis X. J Clin Pathol 35: 327–337PubMedCrossRefGoogle Scholar
  10. 10.
    Ide F, Iwase T, Saito I, Umemura S, Najajima T (1984) Immunohistochemical and ultrastructural analysis of the proliferating cells of histiocytosis X. Cancer 53: 917–921PubMedCrossRefGoogle Scholar
  11. 11.
    Lieberman PH, Jones CR, Dargeon HW, Berg CS (1969) A reappraisal of eosinophilic granuloma of bone, Hand-Schiiller syndrome and Letterer-Siwe syndrome. Medicine 375–400Google Scholar
  12. 12.
    Smith DG, Nesbit ME, D’Angio GJ, Levitt SH (1973) Histiocytosis X. Role of radiation therapy in management with special reference to dose levels employed. Radiol 106: 419–422Google Scholar
  13. 13.
    Nesbit ME, Kieffer S, D’Angio GJ (1969) Reconstitution of vertebral height in histiocytosis X: A long term follow-up. J Bone Joint Surg [Am] 51: 1360–1368Google Scholar
  14. 14.
    Helbock H, Krivit W, Nesbit M: Patterns of antidiuretic function in diabetes insipidus caused by histiocytosis X. J Clin Lab Med 78: 194–202Google Scholar
  15. 15.
    Cohen M, Zorneza J, Cangir A, Murray J A, Wallace S (1980) Direct injection of methyl sodium succinate in the treatment of solitary eosinophilic granuloma of bone. Radiology 136: 289–293PubMedGoogle Scholar
  16. 16.
    Lahey ME (1972) Progress in reticuloendotheliosis in children. J Pediatr 60: 664–671Google Scholar
  17. 17.
    Nesbit ME, Sharp HL, Krivit W (1966) Histiocytosis X. Kelley V (ed) Practice of pediatrics, vol 3. Harper and Row, Hagerstown, pp 1–13Google Scholar
  18. 18.
    Vogel JM, Vogel P (1972) Idiopathic histiocytosis: A discussion of eosinophilic granuloma, Hand- Schiiller-Christian syndrome and Letterer-Siwe syndrome. Semin Hematol 9: 349–369PubMedGoogle Scholar
  19. 19.
    Greenberger JS, Crocker AC, Vawter G, Jaffe N, Cassady RJ (1981) Results of treatment of 127 patients with systemic histiocytosis. Medicine 60: 311–338PubMedCrossRefGoogle Scholar
  20. 20.
    Freud P (1961) Treatment of reticuloendotheliosis. JAMA 175: 82–85PubMedCrossRefGoogle Scholar
  21. 21.
    Starling KA, Donaldson MH, Haggaad ME, Vietti T, Sutow WW (1972) Therapy of histiocytosis with vincristine, vinblastine, and cyclophosphamide. Am J Dis Child 123: 105–110PubMedGoogle Scholar
  22. 22.
    Sharp H, White JG, Krivit W (1964) Histiocytosis X treated with vinblastine (NSC-49842). Cancer Chemother Rep 39: 53–58PubMedGoogle Scholar
  23. 23.
    Jones B, Kung F, Chevalier L (1974) Chemotherapy of reticuloendotheliosis, comparison of methotrexate plus prednisone vs. vincristine plus prednisone. Cancer 34: 1011–1017PubMedCrossRefGoogle Scholar
  24. 24.
    Lahey ME, Heyn RM, Newton WA, Shore N, Smith WB, Leikin S, Hammond D (1979) Histiocytosis X: Clinical trial of chlorambucil: A report from Children’s Cancer Study Group. Med and Pediatr Oncol 7: 197–203CrossRefGoogle Scholar
  25. 25.
    Starling KA (1981) Chemotherapy of histiocytosis. Am J Hematol Oncol 3: 157–163CrossRefGoogle Scholar
  26. 26.
    Lahey ME (1975) Histiocytosis X — comparison of three treatment regimens. J Pediatr 87: 197–183Google Scholar
  27. 27.
    Robb-Smith AHT (1938) Reticulosis and reticulosarcoma; a histologic classification. J Pathol Bacteriol 47: 451CrossRefGoogle Scholar
  28. 28.
    Scott RB, Robb-Smith AHT (1939) Histiocytic medullary reticulosis. Lancet 2: 194–198CrossRefGoogle Scholar
  29. 29.
    Risdall RJ, Sibley RK, McKenna RW, Bevning RD, Dehner LP (1980) Histiocytosis; a light and electron-microscopic and histochemical study. Am J Surg Pathol 4: 439–450PubMedCrossRefGoogle Scholar
  30. 30.
    Risdall RJ, McKenna RW, Nesbit ME, Krivit W, Balfour HH Jr, Simmons RL, Brunning RD (1979) Virus-associated hemophagocytic syndrome; a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 44: 993–1002PubMedCrossRefGoogle Scholar
  31. 31.
    Rubin CM, Burke BA, McKenna RW, McClain KL, Nesbit ME, White JG, Filipovich AH (1983) The virus-associated hemophagocytic syndrome: The cause of the accelerated phase of Chediak-Higashi syndrome. Blood 62: 196Google Scholar
  32. 32.
    Purtilo DT, Sakamoto K, Saemundsen AK, Sullivan JL, Synverholm A, Anvret M, Sloper C, Seeff C, Pincott J, Pachman L, Rich K, Cruze F, Cornet JA, Collins R, Barnes N, Knight J, Sandstedt B, Klein G (1981) Documentation of Epstein-Barr virus infection in immunodeficient patients with life-threatening lymphoproliferative diseases of clinical, virological and immunopathological studies. Cancer Res 41: 4226–4236PubMedGoogle Scholar
  33. 33.
    Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy. Arch Pathol 87: 63–70PubMedGoogle Scholar
  34. 34.
    Flynn KJ, Dehner LP, Gajl-Peszalska KJ (1980) Regressing atypical histiocytosis; a cutaneous proliferation of atypical neoplastic histiocytes with unexpectedly indolent biological behavior. Cancer 49: 959–970CrossRefGoogle Scholar

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© Springer-Verlag Berlin Heidelberg 1986

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  • M. E. Nesbit

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