Important note: Information in this article was accurate in 1987. The state of the art may have changed since the publication date.
CURRENT CONCEPTS AND TREATMENT OF HISTIOCYTOSIS X (LANGERHANS' CELL HISTIOCYTOSIS) IN CHILDREN
Cancer in Children. Clinical Management. Second Edition. Voute PA et al, eds. Current Treatment of Cancer, New York, Springer-Verlag, p. 176-84, 1986.. Unique Identifier : AIDSLINE ICDB/87637339 Nesbit ME; Univ. of Minnesota, Dept. of Pediatrics, Box 484 Mayo Clinic; Bldg., 430 Delaware Street S.E., Minneapolis, MN 55455
Abstract:
Histiocytosis X (Langerhans' cell histiocytosis, LCH) in children is reviewed, including histopathology, characteristics and treatment of localized and generalized disease, and differential diagnosis among various disease entities that are difficult to distinguish from LCH (histiocytic medullary reticulosis, malignant histiocytosis, virus-associated hemophagocytic syndrome, sinus histiocytosis with massive lymphadenopathy, and regressing atypical histiocytosis). The diagnostic cell is the Langerhans' cell, which is characterized by a lobulated and grooved nucleus, a delicate nuclear membrane, unevenly dispersed chromatin, a small nucleolus, and eosinophilic cytoplasm. Localized disease is defined as involving only one organ system. In 21 patients seen at the University of Minnesota from 1960-1973, 19 cases were bone lesions, including 4 with contiguous involvement of lymph node or hypothalamus. Excellent results have been obtained with either curettement and/or radiotherapy. Radiotherapy is recommended when lesions recur or are difficult to remove, and when surgery is contraindicated. Over the same period, 24 children were seen with generalized disease, all of whom had bone lesions, with additional involvement in the skin, liver, lung, and lymph nodes. Beneficial results have been reported with several individual chemotherapeutic agents (eg, corticosteroids, vincristine, vinblastine, chlorambucil, methotrexate, cyclophosphamide, nitrogen mustard, 6-mercaptopurine, and daunorubicin) and with combination chemotherapy. Repeated treatments are the rule, and it usually takes 2-3 yr before the disease becomes inactive. Patients with LCH should be treated with the realization that LCH is not a malignant disease and that the immune (especially T cell) system is closely involved. (34 Refs)
Keywords: Antineoplastic Agents/*THERAPEUTIC USE Antineoplastic Agents, Combined/THERAPEUTIC USE Bone Marrow/PATHOLOGY Child Combined Modality Therapy Histiocytosis, Langerhans-Cell/*DRUG THERAPY/PATHOLOGY/ RADIOTHERAPY Human Langerhans Cells/PATHOLOGY MONOGRAPH
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Important note: Information in this article was accurate in 1987. The state of the art may have changed since the publication date.
