Results of Treatment with 2 - Chlorodeoxyadenosine (2 - CDA) in Multiple Reactivations or Refractory Langerhans Cell Histiocytosis

Abstract

Background: Langerhans cell histiocytosis (LCH) is the most common type of histiocytosis and characterized by abnormal proliferation and excess accumulation of inflammatory and langerhans cells at various tissue sites. Clinical manifestations are variable, ranging from spontaneously regressing single bone lesion to multisystem disease, life-threatening and refractory to treatment. Conventional chemotherapy has been shown to be effective in treatment of majority of patients with LCH. However, treatment of refractory disease or multiple reactivations is difficult. The aim of this study is to assess the efficacy of 2-CDA in relapsed or refractory LCH.