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


Shahla Ansari 1 , Ghasem Miri-Aliabad 2 , * , Khadijeh Arjmandi - Rafsanjani 3


1 Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran

2 Department of Pediatrics, Children and Adolescent Health Research Center, Zahedan University of Medical Sciences, Zahedan, Iran

3 Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran


Zahedan Journal of Research in Medical Sciences: 16 (1); 94-97
Published Online: June 29, 2013
Article Type: Case Report
Received: April 21, 2012
Accepted: July 20, 2012




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.

Materials and Methods: Four patients with relapsed or refractory LCH that were treated with 2-chlorodeoxyadenosin (2-CDA) enrolled in this study. All patients had received at least one prior chemotherapy regimen. The dose and schedule of 2-CDA was 6 mg/m²/day for 5 days every 3-4 weeks.

Results: Median age at the time of treatment with 2-CDA was 9.7 years. Three patients had multisystem disease and one had multifocal bone lesions. All patients had multifocal bone lesions. None of them had risk organ involvement. Mean course of treatment with 2-CDA was 9.5. Radiologic evaluations revealed complete resolution of bone lesions in two (50%) patients. In one (25%) patient lesions regressed (partial response) and in another (25%) the disease remained stable. Drug related side effects were minimal. At the present time all patients are alive.

Conclusion: Our study demonstrates that 2-CDA as a single agent is efficacious in treatment of multiple reactivations or refractory LCH and well-tolerated in children.


Langerhans Cell Histiocytosis 2-Chlorodeoxyadenosine Children

© 0, Zahedan Journal of Research in Medical Sciences. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License ( which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.

Fulltext is availale in the PDF.

  • 1. References are available in the PDF.