Please use this identifier to cite or link to this item: http://ahro.austin.org.au/austinjspui/handle/1/12305
Title: High-dose methotrexate for the treatment of relapsed central nervous system erdheim-chester disease.
Authors: Ho, Prahlad;Smith, Carole
Affiliation: Department of Clinical Haematology, Austin Health, Heidelberg, VIC, Australia.
Issue Date: 16-Jun-2014
Citation: Case Reports in Hematology 2014; 2014(): 269359
Abstract: Erdheim-Chester disease (ECD) is a rare multisystem non-Langerhans histiocytosis. CNS involvement is a major complication, which is often rapidly progressive and confers a poor prognosis. However, treatment of CNS ECD is difficult due to poor CNS penetrance by the most effective chemotherapeutic drugs commonly used in this disorder (e.g., interferon and cladribine). We describe a case of a 60-year-old lady with a 5-year history of stable systemic ECD who presented with new brainstem lesions and rapid, steroid-refractory neurological deterioration which required immediate intervention. High-dose methotrexate was chosen due to its rapid onset of action and excellent CNS penetration. Her neurological deterioration was quickly arrested with significant functional improvement, which was sustained for 4 months with consolidation doses of high-dose methotrexate. Subsequent treatment with cladribine and interferon did not confer any appreciable clinical improvement. High-dose methotrexate is effective in controlling rapidly progressive CNS ECD and should be considered as a salvage agent prior to commencement of more definitive treatment.
Internal ID Number: 25031876
URI: http://ahro.austin.org.au/austinjspui/handle/1/12305
DOI: 10.1155/2014/269359
URL: http://www.ncbi.nlm.nih.gov/pubmed/25031876
Type: Journal Article
Appears in Collections:Journal articles

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