Childhood Histiocytosis X: Clinical Aspects and Therapeutic Approaches
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.
KeywordsDiabetes Insipidus Hematopoietic System Eosinophilic Granuloma Malignant Histiocytosis Host Tolerance
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