LOW-DOSE ORAL ETOPOSIDE MONOTHERAPY IN ADULT LANGERHANS CELL HISTIOCYTOSIS

Background: The purpose of this study was to test the disease-controll ing effect of low-dose oral etoposide monotherapy in adult-onset multi system Langerhans cell histiocytosis. There are no previous reports of low-dose etoposide monotherapy for this condition. Observations: A 27 -year-old man with a 7-year history of multifocal chronic Langerhans c ell histiocytosis presented with severe disabling ulcers in intertrigi nous areas. He had previously been treated with 2 different regimens o f antitumoral chemotherapy; one had to be discontinued due to myelosup pression and the other had proved ineffective. We treated with oral et oposide monotherapy at 50 mg/d (22 mg/m(2) per day) for 21 days. The t reatment was repeated at 28-day intervals for a total of 6 cycles. A r apid initial response with subtotal diminution of the involved skin ar ea was found. No adverse effects were observed. The clinical picture h as remained stable during the 7 months following cessation of therapy. Conclusion: Low-dose oral etoposide treatment is an adequate therapeu tic measure for prolonged disease control in adult-type Langerhans cel l histiocytosis.