Treatment of adult Langerhans cell histiocytosis with etoposide.

Abstract

Langerhans cell histiocytosis (LCH) is a reactive disease in which abnormal Langerhans cells accumulate in various body sites and cause damage to affected organs. Adults often do not respond to conventional therapy with local or systemic steroids. Our purpose was to investigate intravenous etoposide as monotherapy in the treatment of adult patients with severe or resistant LCH. In an open study, three adult patients with LCH (one with single-system skin disease and two with multisystem disease) were treated with etoposide, 100 mg/m2/day, for 3 days. This was repeated every 3 or 4 weeks for three or four cycles. All patients achieved clinical remission that persisted during a 12- to 14-month follow-up. No serious side effects were noted. We recommend the use of etoposide monotherapy in severe or restricted LCH in adults.