Childhood Histiocytosis X: Clinical Aspects and Therapeutic Approaches

  • A. J. Feldges
Part of the Haematology and Blood Transfusion / Hämatologie und Bluttransfusion book series (HAEMATOLOGY, volume 27)


Childhood histiocytosis X is a disorder of the monocyte-macrophage system with various clinical aspects and immunological abnormalities. The disease may be localized or disseminated, with an acute, subacute, or chronic course which can lead to death or many residual damages. Therapy depends on the extent of the disease and risk factors such as involvement of the lung, the liver, and the hematopoietic system. For localized lesions in the bone no therapy except surgical curettage is necessary. In radiotherapy a dose of 600 rad is usually adequate; the role of radiotherapy as opposed to treatment with surgical measures should be further investigated. For disseminated forms chemotherapy is the treatment of choice. Immunotherapy with thymic extract may be an alternative but must be studied further in the future. The response to specific therapy may be slow; modification or discontinuation depends on host tolerance and toxicity. Prospective controlled trials considering the natural history of the disease, immunological aspects and risk criteria are warranted.


Diabetes Insipidus Hematopoietic System Eosinophilic Granuloma Malignant Histiocytosis Host Tolerance 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Elema JD, Poppema S (1978) Infantile histiocytosis X (Letterer Siwe disease). Investigation with enzyme histochemical and sheep erythrocyte rosetting techniques. Cancer 42: 555PubMedCrossRefGoogle Scholar
  2. 2.
    Feldges A, Meuret G, Gonzenbach P, Fust G (1979) Histiocytosis X in 9 children: clinical aspects and laboratory evaluations including an analysis of monocytopoiesis. Helv Paediatr Acta 34: 107PubMedGoogle Scholar
  3. 3.
    Feldges AJ, Imbach P, Plüss HJ, Sartorius J, Wagner HP, Wyss M (1980) Therapie der disseminierten Histiocytosis X. Schweiz Med Wochenschr 110: 912PubMedGoogle Scholar
  4. 4.
    Jones B, Kung F, Chevalier L, Forman EN, Rausen A, Koch K, Despoto F, Maurer H, Jacquillat C, Degnan TJ, Pluess HJ, Desorges J, Patterson RB, Glidewell O, Holland J (1974) Chemotherapy of reticuloendotheliosis. Comparison of methotrexate + prednisone. Cancer 34: 1011PubMedCrossRefGoogle Scholar
  5. 5.
    Komp DM, Vietti TJ, Berry DH, Starling KA, Haggard ME, George SL (1977) Combination che-motherapy in histiocytosis X. Med Pediatr Oncol 3: 267PubMedCrossRefGoogle Scholar
  6. 6.
    Lahey ME (1962) Prognosis in reticuloendotheliosis in children. J Pediatr 60: 664CrossRefGoogle Scholar
  7. 7.
    Lahey ME (1975) Histiocytosis X — an analysis of prognostic factors. J Pediatr 87: 184PubMedCrossRefGoogle Scholar
  8. 8.
    Lahey ME (1975) Histiocytosis X — comparison of three treatment regimens. J Pediatr 87: 179PubMedCrossRefGoogle Scholar
  9. 9.
    Lahey ME, Heyn RM, Newton WA, Shore N, Smitz WB, Leikin S, Hammond D (1979) Histiocytosis X — Clinical trial of chlorambucil: A report from Children’s Cancer Study Group. Med Pediatr Oncol 7: 197PubMedCrossRefGoogle Scholar
  10. 10.
    Lichtenstein L (1953) Histiocytosis X. Integration of eosinophilic granuloma of bone, “Letterer Siwe disease” and “Schüller Christian disease” as related manifestations of a single nosologic entity. Arch Pathol 56: 84Google Scholar
  11. 11.
    Liebermann PH, Jones CR, Dargeon HWK, Begg CF (1969) A reappraisal of eosinophilic granu-loma of bone, Hand Schüller Christian syndrome and Letterer Siwe syndrome. Medicine (Baltimore) 48: 375Google Scholar
  12. 12.
    Nezelof C, Frileux-Herbet F, Cronier-Sachot J (1979) Disseminated histiocytosis X. Analysis of prognostic factors based on a retrospective study of 150 cases. Cancer 44: 1824PubMedCrossRefGoogle Scholar
  13. 13.
    Osband M, Lipton J, Vawter G, Levey R, Parkman R (1978) Treatment of histiocytosis X with calf thymus extract. Blood 50: 268Google Scholar
  14. 14.
    Sims DG (1977) Histiocytosis X. Follow-up of 43 cases. Arch Dis Child 52: 433PubMedCrossRefGoogle Scholar
  15. 15.
    Smith PJ, Ekert H, Campbell PE (1976) Improved prognosis in disseminated histiocytosis. Med Oncol 2: 371CrossRefGoogle Scholar
  16. 16.
    Starling KA, Donaldson MH, Haggard ME, Vietti TE, Sutow WW (1972) Therapy of histiocytosis X with vincristine, vinblastine and cyclophosphamide. Am J Dis Child 123: 105PubMedGoogle Scholar
  17. 17.
    Vogel J, Vogel P (1972) Idiopathic histiocytosis: a discussion of eosinophilic granuloma, the Hand Schüller Christian syndrome and the Letterer Siwe syndrome. Semin Hematol 9: 349PubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg New York 1981

Authors and Affiliations

  • A. J. Feldges

There are no affiliations available

Personalised recommendations