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Most investigators have previously recommended treatment according to the guidelines given above for standard treatment of children with Langerhans cell histiocytosis. It is unclear, however, whether adult LCH responds as well as the childhood form of the disease. In addition, the drugs used in the treatment of children appear to be less well-tolerated in adults. Excessive neurologic toxicity from vinblastine, for example, prompted closure of the LCH-A1 trial.
Treatment of pulmonary LCH
It is difficult to judge the effectiveness of various treatments for pulmonary LCH as patients can recover spontaneously or have stable disease without treatment. Smoking cessation is mandatory in view of the apparent causal effect of smoking in pulmonary LCH. It is not known if steroid therapy is efficacious in the treatment of adult pulmonary LCH because reported case series did not control for smoking cessation. Most adult patients with LCH have gradual disease progression with continued smoking. The disease may regress or progress with the cessation of smoking.
Lung transplant may be necessary for adults with extensive pulmonary destruction from LCH. This multicenter study reported 54% survival at 10 years posttransplant with 20% of patients having recurrent LCH that did not impact survival; longer follow-up of these patients is needed. The best strategy for follow-up of pulmonary LCH includes physical examination, chest radiographs, lung function tests, and high-resolution computed tomography (CT) scans.
Treatment of bone LCH
Similar to children, adults with single-bone lesions should undergo curettage of the lesion followed by observation, with or without intralesional corticosteroids. Extensive or mutilating surgery is contraindicated at any site, including the teeth or jaw bones. Systemic chemotherapy will cause bone lesions to regress and the involved teeth and jaw bones cannot reform. For those failing chemotherapy, low-dose radiation therapy may be indicated and should be tried prior to any mutilating surgery. Radiation therapy is also indicated for impending neurological deficits from vertebral body lesions or visual problems from orbital lesions.
A variety of chemotherapy regimens, including 2-CdA have been published in a relatively limited number of patients. (Refer to the Chemotherapy section of this summary for more information.)
Anecdotal reports have described the successful use of the bisphosphonate pamidronate in controlling severe bone pain in patients with multiple osteolytic lesions.[4,5,6] Successful use of oral bisphosphonates have also been described and may be a useful and relatively low-toxic way of treating adult bone LCH. In view of the increased toxicity of chemotherapy in adults, bisphosphonate therapy could be used prior to chemotherapy in multifocal bone disease. Response of other organs, such as skin and soft tissue, to bisphosphonate therapy has been reported.