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

Shahla  Ansari; Ghasem Miri-Aliabad; Khadijeh Arjmandi - Rafsanjani

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

Author(s):

Shahla Ansari¹,

Ghasem Miri-Aliabad^2,*,

Khadijeh Arjmandi - Rafsanjani³

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

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

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

Zahedan Journal of Research in Medical Sciences:Vol. 16, issue 1; 94-97

Published online:Jun 29, 2013

Article type:Case Report

Received:Apr 21, 2012

Accepted:Jul 20, 2012

How to Cite:Shahla AnsariGhasem Miri-AliabadKhadijeh Arjmandi - RafsanjaniResults of Treatment with 2 - Chlorodeoxyadenosine (2 - CDA) in Multiple Reactivations or Refractory Langerhans Cell Histiocytosis.Zahedan J Res Med Sci.16(1):e1769.

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.

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.

Keywords

Langerhans Cell Histiocytosis

2-Chlorodeoxyadenosine

Children

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