Successful Treatment of Langerhans'-Cell Histiocytosis with Etanercept

Abstract

Langerhans'-cell histiocytosis, previously called histiocytosis X, is an accumulation of dendritic Langerhans' cells and granulomatous lesions in various organs; its cause is unknown. The three-year survival rate among children with Langerhans'-cell histiocytosis has not improved during the past two decades and remains around 80 percent.¹ In children younger than two years old who have involvement of the liver, spleen, hematopoietic system, or lungs and who do not have a response to induction therapy over the course of six weeks, the probability of survival at three years is less than 20 percent.¹ We describe a child with Langerhans'-cell histiocytosis that progressed during conventional treatment but responded to treatment with an agent (etanercept) that neutralizes tumor necrosis factor α (TNF-α).