Adult Langerhans' Cell Histiocitosis (LCH). A Single Institution Experience.

Abstract

Langerhans' cell histiocytosis (LCH) is a rare clonal disorder that affects mainly children. Clinical presentation as well as behaviour are heterogeneous and can range from solitary, relatively benign lesions to a fatal disseminated disease. Although the features have been well described in children, they remain poorly defined in adults. We report the clinical characteristics and outcome of 30 adults from a single institution.Clinical presentation: From 1957 to 2003, 30 patients were diagnosed with LCH at MDA. Median age at presentation was 37 years-old, with 60% of being male. Depending on the organs involved and according to the pediatric classification, 17 cases (57%) had a limited form of LCH (single-system) and 13 cases (43%) had disseminated disease (multisystemic). Patients with single-system LCH had mainly bone (76%) and skin (24%) involvement, which was localized to a single site in 10 cases (58%) and multifocal in the remaining. Patients with limited disease were younger (median age 30 yo). Patients with multisystemic LCH were subdivided into a “high risk” category (5 patients) if lung, liver, spleen, or bone marrow was involved, or into a “low risk” category (8 cases), if none of the risk organs was affected. Six patients had pulmonary involvement, typically as bilateral small nodules although diffuse progressive infiltrates were also present in 2 cases.Clinical course: Patients with limited disease were mainly treated with steroids, radiation, or immunomodulatory agents while those with multifocal or multi-systemic disease had a more agressive behaviour and were treated with systemic therapy. Patients with pulmonary nodules usually responded to systemic steroids and stop of smoking habit. Median of 3 treatments were given (range 1 to 7). Sixty-one percent of patients had a 30-year overall survival (OS). Single-system LCH had a more indolent course (79% OS at 30 years) than multi-systemic LCH (median OS of 16 years). Risk organ involvement was the most important adverse prognostic factor (median OS of 2.2 years vs. 65% OS at 30 years, p: 0.007). [Display omitted] [Display omitted]