{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,25]],"date-time":"2026-03-25T11:29:19Z","timestamp":1774438159717,"version":"3.50.1"},"reference-count":19,"publisher":"American Society of Hematology","issue":"10","content-domain":{"domain":["ashpublications.org"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2011,3,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-\u03b1 treatment would influence survival in a large cohort of ECD patients. To achieve this goal, we thoroughly analyzed the clinical presentation of 53 patients with biopsy-proven ECD, and we performed a survival analysis using Cox proportional hazard model. Fifty-three patients (39 men and 14 women) with biopsy-proven ECD were followed up between November 1981 and November 2010. Forty-six patients (87%) received interferon-\u03b1 and\/or PEGylated interferon-\u03b1. Multivariate survival analysis using Cox proportional hazard model revealed that central nervous system involvement was an independent predictor of death (hazard ratio = 2.51; 95% confidence interval, 1.28-5.52; P = .006) in our cohort. Conversely, treatment with interferon-\u03b1 was identified as an independent predictor of survival (hazard ratio = 0.32; 95% confidence interval, 0.14-0.70; P = .006). Although definitive confirmation would require a randomized controlled trial, these results suggest that interferon-\u03b1 improves survival in ECD patients. This may be seen as a significant advance, as it is the first time a treatment is shown to improve survival in this multisystemic disease with high mortality.<\/jats:p>","DOI":"10.1182\/blood-2010-06-294108","type":"journal-article","created":{"date-parts":[[2011,1,15]],"date-time":"2011-01-15T03:40:04Z","timestamp":1295062804000},"page":"2778-2782","update-policy":"https:\/\/doi.org\/10.1182\/blood.2019cm0000","source":"Crossref","is-referenced-by-count":330,"title":["CNS involvement and treatment with interferon-\u03b1 are independent prognostic factors in Erdheim-Chester disease: a multicenter survival analysis of 53 patients"],"prefix":"10.1182","volume":"117","author":[{"given":"Laurent","family":"Arnaud","sequence":"first","affiliation":[{"name":"Department of Internal Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases, AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re H\u00f4pital, Paris, France;"},{"name":"Inserm, UMR, S 945, Paris, France;"}]},{"given":"Baptiste","family":"Hervier","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases, AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re H\u00f4pital, Paris, France;"},{"name":"UPMC Univ Paris 06, Paris, France;"}]},{"given":"Antoine","family":"N\u00e9el","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, CHU H\u00f4tel-Dieu, Nantes, France;"}]},{"given":"Mohamed A.","family":"Hamidou","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, CHU H\u00f4tel-Dieu, Nantes, France;"}]},{"given":"Jean-Emmanuel","family":"Kahn","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, H\u00f4pital Foch, Suresnes, France;"}]},{"given":"Bertrand","family":"Wechsler","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases, AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re H\u00f4pital, Paris, France;"}]},{"given":"Gemma","family":"P\u00e9rez-Pastor","sequence":"additional","affiliation":[{"name":"Department of Dermatology, La Plana de Villarreal Hospital, Castell\u00f3n, Spain;"}]},{"given":"Bj\u00f8rn","family":"Blomberg","sequence":"additional","affiliation":[{"name":"Institute of Internal Medicine, University of Bergen, Bergen, Norway;"}]},{"given":"Jean-Gabriel","family":"Fuzibet","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, H\u00f4pital de l'Archet, CHU, Nice, France;"}]},{"given":"Fran\u00e7ois","family":"Dubourguet","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, HIA Val-de-Gr\u00e2ce, Paris, France;"}]},{"given":"Ant\u00f3nio","family":"Marinho","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Hospital de Santo Ant\u00f3nio, Centro Hospitalar do Porto, Porto, 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Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases, AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re H\u00f4pital, Paris, France;"}]},{"given":"Laurent","family":"Aaron","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, H\u00f4pital Jacques Coeur, Bourges, France;"}]},{"given":"Juan","family":"Salvatierra","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, University Hospital Viergen de las Nieves, Granada, Spain;"}]},{"given":"Carlos","family":"Graux","sequence":"additional","affiliation":[{"name":"Department of Hematology, Cliniques Universitaires St Luc, Catholic University of Louvain, Brussels, Belgium;"}]},{"given":"Patrice","family":"Cacoub","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and French Reference Center for Rare Autoimmune and Systemic Diseases, AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re H\u00f4pital, Paris, France;"}]},{"given":"V\u00e9ronique","family":"Delcey","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, CHU Lariboisi\u00e8re, Paris, France;"}]},{"given":"Claudia","family":"Dechant","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Medizinische Poliklinik, University of Munich, Munich, Germany;"}]},{"given":"Pascal","family":"Bindi","sequence":"additional","affiliation":[{"name":"Department of Medicine A, Centre Hospitalier de Verdun, Verdun, France;"}]},{"given":"Christiane","family":"Herbaut","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Endocrinology, CHU Brugmann, Bruxelles, Belgium; and"}]},{"given":"Giorgio","family":"Graziani","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine and Nephrology, Istituto Clinico Humanitas IRCCS, Rozzano-Milano, Italy"}]},{"given":"Zahir","family":"Amoura","sequence":"additional","affiliation":[{"name":"Department of Internal 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