MiscellaneousTreatment of Erdheim-Chester Disease with Long-Term High-Dose Interferon-α
Section snippets
Patients
This study was based on the prospective follow-up of all ECD patients (n = 27) who received high doses of IFNα, ie, either ≥18 mIU/wk (3 injections weekly) or PEG-IFNα ≥180 μg/wk (1 injection weekly), at La Pitié-Salpêtrière Hospital (Paris, France), a tertiary care center, from April 1995 to January 2011. In all cases, ECD diagnosis was based on typical histologic features: infiltration with foamy histiocytes nested among polymorphic granuloma and fibrosis with CD68-positive and CD1a-negative
Patients
Twenty-four patients (17 men and 7 women) were included in this prospective study. The median age at diagnosis was 59 years (range, 26-77 years). Patient characteristics are summarized in Table 1. These patients were treated with IFNα 18 mIU/wk (n = 3), IFNα 27 mIU/wk (n = 11), or PEG-IFNα 180 μg/wk (n = 10).
High doses of IFNα were used as first-line (n = 9) or second-line treatment (n = 15). Of the 15 patients who received IFNα as a second-line treatment, 12 had previously received
Discussion
ECD is a slowly evolving histiocytosis and improvement under treatment usually leads only to partial remission, rather than complete recovery (7, 10, 11, 13, 15). Thus, evaluation of ECD may be challenging and requires large cohorts with a long-term follow-up. We have previously reported variable efficacy of standard-dose IFNα in a larger series of severe ECD patients (11). However, at standard IFNα doses, partial remissions depended on the sites of ECD involvement (11). We therefore conducted
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The authors have no conflicts of interest to disclose.