Abstract
Langerhans cell histiocytosis (LCH) is rare in adults, and only a subset of these patients suffers from central nervous system (CNS) involvement. Hence, evidence-based treatment recommendations are lacking. A case of a 20-year-old student with multisystem LCH and extensive CNS involvement is described, who showed a durable response to 2-chlorodeoxyadenosine after prior therapies with the tyrosine kinase inhibitors sorafenib and imatinib. In accordance to the experiences provided by other case series, which are reviewed herein, 2-chlorodeoxyadenosine can be considered an effective and safe option for adult LCH with CNS involvement.
Copyright © 2011 John Wiley & Sons, Ltd.
MeSH terms
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Adult
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Antineoplastic Agents / therapeutic use*
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Benzamides
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Benzenesulfonates / administration & dosage*
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Central Nervous System Diseases / complications
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Central Nervous System Diseases / drug therapy*
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Cladribine / therapeutic use*
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Histiocytosis, Langerhans-Cell / drug therapy*
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Histiocytosis, Langerhans-Cell / etiology
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Humans
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Imatinib Mesylate
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Niacinamide / analogs & derivatives
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Phenylurea Compounds
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Piperazines / administration & dosage*
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Protein Kinase Inhibitors / administration & dosage*
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Pyridines / administration & dosage*
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Pyrimidines / administration & dosage*
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Sorafenib
Substances
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Antineoplastic Agents
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Benzamides
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Benzenesulfonates
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Phenylurea Compounds
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Piperazines
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Protein Kinase Inhibitors
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Pyridines
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Pyrimidines
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Niacinamide
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Cladribine
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Imatinib Mesylate
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Sorafenib