LETTER

Treatment of Erdheim-Chester disease with cladribine: a rational approach

C Myra¹, L Sloper¹, P J Tighe^1,2, R S McIntosh^2,3, S E Stevens², R H S Gregson⁴, M Sokal⁵, A P Haynes⁶ and R J Powell⁷

¹ Division of Ophthalmology, University of Nottingham School of Clinical Laboratory Sciences, Queen’s Medical Centre, Nottingham NG7 2UH, UK
² Division of Immunology, University of Nottingham School of Clinical Laboratory Sciences, Queen’s Medical Centre, Nottingham NG7 2UH, UK
³ Division of Ophthalmology, University of Nottingham School of Clinical Laboratory Sciences, Queen’s Medical Centre, Nottingham NG7 2UH, UK
⁴ Department of Diagnostic Imaging, Queen’s Medical Centre, Nottingham NG7 2UH, UK
⁵ Department of Oncology, City Hospital, Nottingham NG5 1PB, UK
⁶ Department of Haematology, City Hospital, Nottingham NG5 1PB, UK
⁷ Clinical Immunology Unit, Queen’s Medical Centre, Nottingham NG7 2UH, UK

Correspondence to:
Correspondence to:
C Myra L Sloper
Neuro-Ophthalmology Department, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK; myra.sloper@dial.pipex.com

Accepted 5 November 2003

Keywords: Erdheim-Chester disease; cladribine; orbital pseudotumour; histiocytosis

The first 150 words of the full text of this article appear below.

Erdheim-Chester disease is a rare, life threatening lipoid granulomatosis¹ with fewer than 100 cases described in the world literature. The disease typically affects the long bones and symmetrical sclerosis of the diaphyseal and metaphyseal regions is pathognomonic. Extraskeletal manifestations may affect the lungs, pericardium, aorta, retroperitoneum, skin, and orbits and diabetes insipidus occurs in approximately 30% of cases. Erdheim-Chester disease is characterised microscopically by an infiltrate of lipid laden foamy macrophages (histiocytes), scattered Touton giant cells, chronic inflammatory cells, and fibrosis. The foamy macrophages can be distinguished from Langerhans cells on the basis of negative results on staining for S-100 protein and CD1a. Treatment of the disease has been on an ad hoc basis and no treatment regimen has been shown to be clearly superior.

This study documents the clinical findings in a patient with Erdheim–Chester disease, investigates the pathogenesis, and provides a rational basis for effective treatment.

This white . . . [Full text of this article]

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• Braiteh, F., Boxrud, C., Esmaeli, B., Kurzrock, R. (2005). Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-{alpha}. Blood 106: 2992-2994 [Abstract] [Full Text]