TREATMENT OF LANGERHANS CELL HISTIOCYTOSIS WITH 2-CHLORODEOXYADENOSINE

Langerhans-cell histiocytosis (LCH) is a rare condition with a wide cl inical spectrum and variable prognosis. Patients with multisystem LCH have been treated with a variety of agents but may develop resistant a nd progressive disease. Based on a preliminary encouraging report on t he activity of 2 chlorodoxyadenosine in this disease, we administered this agent to a patient with LCH which was resistant to corticosteroid s and etoposide. After 4 courses of treatment the patient achieved a c omplete remission which is currently ongoing for 12 months. 2 CdA appe ars to be effective in patients with resistant LCH and warrants invest igation in previously untreated patients with poor risk disease.