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Blood, 1 November 2005, Vol. 106, No. 9, pp. 2992-2994. Prepublished online as a Blood First Edition Paper on July 14, 2005; DOI 10.1182/blood-2005-06-2238. This Article Full Text (PDF) All Versions of this Article: 2005-06-2238v1 106/9/2992    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Braiteh, F. Articles by Kurzrock, R. Search for Related Content PubMed PubMed Citation Articles by Braiteh, F. Articles by Kurzrock, R. Related Collections Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted June 3, 2005 Accepted June 17, 2005 Successful treatment of Erdheim-Chester disease,a non-Langerhans cell histiocytosis, with interferon-alpha Fadi Braiteh, Cynthia Boxrud, Bita Esmaeli, and Razelle Kurzrock* Phase I Program, Division of Cancer Medicine and the University of Texas Graduate School of Biomedical Sciences, Houston, Texas, USA Jules Stein Eye Institute, UCLA, Santa Monica, California, USA Section of Ophthalmology, Department of Plastic Surgery, MD Anderson Cancer Center, Houston, Texas, USA * Corresponding author; email: rkurzroc{at}mdanderson.org. Erdheim-Chester disease is a rare non-Langerhans histiocytosis with multisystem involvement. To date, there is no standard treatment for this disorder, and over half of the patients succumb within three years. Because interferon-alpha promotes the terminal differentiation of histiocytes and dendritic cells, we hypothesized that this molecule would be a useful therapy for Erdheim-Chester disease. We therefore treated three patients with advanced disease with interferon-alpha at a starting dose of 3 to 6 x106 units, which was later reduced, during maintenance, to 1 x106 units s.c. three times per week. Marked improvement was noted in all patients, with substantial retro-orbital disease regression within one month. Improvement in bone lesions, pain, diabetes insipidus and other manifestations was gradual over many months. Responses were durable (3+ to 4.5+ years). Our observations suggest that this well-tolerated therapy has a significant impact on the course and outcome of Erdheim-Chester disease. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Interferon-: still useful after all these years Eric J. Feldman Blood 2005 106: 2930-2931. [Full Text] [PDF] This article has been cited by other articles: J. A. Mills, R. G. Gonzalez, and R. Jaffe Case 25-2008 -- A 43-Year-Old Man with Fatigue and Lesions in the Pituitary and Cerebellum N. Engl. J. Med., August 14, 2008; 359(7): 736 - 747. [Full Text] [PDF] J. Haroche, Z. Amoura, F. Charlotte, J. Salvatierra, B. Wechsler, C. Graux, N. Brousse, and J.-C. Piette Imatinib mesylate for platelet-derived growth factor receptor-beta-positive Erdheim-Chester histiocytosis Blood, June 1, 2008; 111(11): 5413 - 5415. [Full Text] [PDF]

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