Successful Treatment of Langerhans'-Cell Histiocytosis with Etanercept

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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).