Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-α

Fadi Braiteh, Cynthia Boxrud, Bita Esmaeli, Razelle Kurzrock

Research output: Contribution to journalArticle

143 Citations (Scopus)

Abstract

Erdheim-Chester disease is a rare non-Langerhans histiocytosis with multisystem involvement. To date, there is no standard treatment for this disorder, and more than half of the patients succumb within 3 years. Because interferon-α 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 3 patients with advanced disease with interferon-α at a starting dose of 3 to 6 × 10 6 units, which was later reduced, during maintenance, to 1 × 106 units subcutaneous 3 times per week. Marked improvement was noted in all patients, with substantial retro-orbital disease regression within 1 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 effect on the course and outcome of Erdheim-Chester disease.

Original languageEnglish (US)
Pages (from-to)2992-2994
Number of pages3
JournalBlood
Volume106
Issue number9
DOIs
StatePublished - Nov 1 2005

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Non-Langerhans-Cell Histiocytosis
Erdheim-Chester Disease
Interferons
Orbital Diseases
Histiocytosis
Diabetes Insipidus
Histiocytes
Dendritic Cells
Therapeutics
Maintenance
Bone and Bones
Pain
Medical problems
Bone
Molecules

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-α. / Braiteh, Fadi; Boxrud, Cynthia; Esmaeli, Bita; Kurzrock, Razelle.

In: Blood, Vol. 106, No. 9, 01.11.2005, p. 2992-2994.

Research output: Contribution to journalArticle

Braiteh, Fadi ; Boxrud, Cynthia ; Esmaeli, Bita ; Kurzrock, Razelle. / Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-α. In: Blood. 2005 ; Vol. 106, No. 9. pp. 2992-2994.
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