Cladribine in the Treatment of Langerhans Cell Histiocytosis: A Summary of the Literature

Marlies E.H.M Van Hoef

doi:10.21767/2380-7245.100012

Abstract

Langerhans cell Histiocytosis (LCH) is a rare disease for which treatment with 2- chlorodeoxyadenosine (cladribine) has changed disease outcome. Cladribine has meanwhile been administered over two decades and is available for intravenous and subcutaneous administration. Cladribine has been administered in treatment of therapy naïve, relapsed and refractory LCH and induces high response rates, regardless of prior treatment. Literature describing the use of cladribine either alone or in combination with cytarabine is summarized herein.

Highlights

Langerhans cell histiocytosis (LCH) is a rare disease involving clonal proliferation of Langerhans cells, which are abnormal cells derived from the bone marrow, along with eosinophils, macrophages, lymphocytes and multinucleated giant cells
In the guidelines for children up to eighteen years with LCH, cladribine monotherapy is recommended in first line treatment for LCH of the lung and LCH brain lesions [3]
Cladribine monotherapy is recommended as second line treatment in LCH with low risk organ involvement and in combination with cytarabine when high risk organs are involved [3]

Summary

Introduction

LCH is a rare disease involving clonal proliferation of Langerhans cells, which are abnormal cells derived from the bone marrow, along with eosinophils, macrophages, lymphocytes and multinucleated giant cells. In the management recommendations for adult patients with LCH cladribine monotherapy is recommended in front line treatment of systemic therapy of single-system LCH with special site lesions, multifocal lesions and for multi-system LCH with or without involvement of high risk organs [4]. Cladribine is recommended as treatment option when maintenance therapy is required, in refractory disease with or without cytarabine and in single or multisystem reactivation of LCH and in central nervous system involvement, regardless whether there are tumorous lesions or neurodegenerative disease [4]. Cladribine (2-chlorodeoxyadenosine) is a purine analog that was developed in the 1970s It was first tested in humans in the early 1980s and became an established product for the treatment of Langerhans cell histiocytosis (LCH). A summary of the efficacy of cladribine in LCH is described in this paper

Methods

PR 1 durable CR

Kids 4 adults

Findings

Discussion