TREATMENT OF LANGERHANS-CELL HISTIOCYTOSIS IN CHILDREN - EXPERIENCE AT THE CHILDRENS-HOSPITAL OF NANCY

Forty children, who had Langerhans-cell histiocytosis were followed fo r an average of six: Sears (range, excluding patients who died of the disease, two to fifteen gears). The patients were divided into two dia gnostic groups: those who had localized disease (involving one bone or more only) and those who had multifocal disease (an osseous lesion an d a soft-tissue mass, a skin rash, diabetes insipidus, or generalized disease). Methods of treatment included curettage, bone grafting, chem otherapy, local or systemic corticosteroids, and radiotherapy. Ninetee n of the thirty patients who had localized disease had a complete resp onse to the therapy four had a partial response, and seven had no resp onse. Twenty-one of these thirty patients had not had a recurrence by the time of the latest follow-up examination; nine had a local recurre nce within four years after the initial therapy but had no additional recurrences after treatment of the local recurrence. No recurrence occ urred more than four years after the time that the initial diagnosis h ad been made. Five of the ten patients who had multifocal disease had a complete response to the therapy, two had a partial response, and th ree had no response. Six patients had a recurrence; four did not. Two patients died of the disease. As a result of this study, we recommend the avoidance of intensive measures of treatment, if possible, and we advise long-term follow-up of these patients.