LANGERHANS-CELL HISTIOCYTOSIS IN ADULTS

Guided by a long-term retrospective observation, the clinical course a nd treatment of Langerhans'-cell histiocytosis (ICH) in adult patients are represented. The series included 19 patients meeting the histopat hologic criteria of presumptive LCH who were followed for 1.5-20 years (average 7.7 years). Most frequently skeletal lesions (16 patients), diffuse interstitial lung infiltrates (seven patients), and pituitary gland involvement with diabetes insipidus (four patients) were present . Bone lesions of the skull and axial skeleton were associated with an infiltration of adjacent soft tissues in 10 of 16 patients. Liver, ly mph node, and bone marrow involvement appeared sporadically. LCH was d ivided into localized or multifocal form. Localized disease took a ben ign course with remission of bone (n = 4) or lymph node lesions (n = 2 ). Also, in isolated pulmonary LCH (n = 2), spontaneous transition to inactive disease occurred. With the exception of isolated bone lesions (n = 27), which remained asymptomatic or showed a remission to treatm ent, multifocal LCH had a more aggressive course. Osseous lesions with adjacent soft tissue infiltration (n = 20) showed a relapse rate in e xcess of 80% independent of the treatment applied. Pulmonary involveme nt led to a more marked functional impairment compared to the isolated form, and systemic treatment yielded no convincing effect, in three p atients with liver or bone marrow involvement, LCH showed a persistent , serious disease activity. One patient died of transition into acute monomyelocytic leukemia 18 months after diagnosis without preceding ch emotherapy. In adults, LCH seems to be limited to a few organ systems. Multifocal LCH represents the more aggressive form with unfavorable p rognosis in patients with bone lesions spreading into the adjacent sof t tissue and liver or bone marrow involvement. (C) 1997 Wiley-Liss, In c.