Guided by a long-term retrospective observa-bone lesions (n ฯญ 27), which remained asymption, the clinical course and treatment of Langer-tomatic or showed a remission to treatment, hans'-cell histiocytosis (LCH) in adult patients multifocal LCH had a more aggressive course. are represented. The series included 19 patients Osseous lesions with adjacent soft tissue infiltrameeting the histopathologic criteria of presump-tion (n ฯญ 20) showed a relapse rate in excess tive LCH who were followed for 1.5-20 years of 80% independent of the treatment applied. (average 7.7 years).
Pulmonary involvement led to a more marked Most frequently, skeletal lesions (16 patients), functional impairment compared to the isolated diffuse interstitial lung infiltrates (seven pa-form, and systemic treatment yielded no contients), and pituitary gland involvement with dia-vincing effect. In three patients with liver or bone betes insipidus (four patients) were present. marrow involvement, LCH showed a persistent, Bone lesions of the skull and axial skeleton were serious disease activity. One patient died of tranassociated with an infiltration of adjacent soft sition into acute monomyelocytic leukemia 18 tissues in 10 of 16 patients. Liver, lymph node, months after diagnosis without preceding cheand bone marrow involvement appeared spo-motherapy. radically.
In adults, LCH seems to be limited to a few LCH was divided into localized or multifocal organ systems. Multifocal LCH represents the form. Localized disease took a benign course more aggressive form with unfavorable prognowith remission of bone (n ฯญ 4) or lymph node sis in patients with bone lesions spreading into lesions (n ฯญ 2). Also, in isolated pulmonary LCH the adjacent soft tissue and liver or bone marrow (n ฯญ 2), spontaneous transition to inactive dis-involvement.
แฎ 1997 Wiley-