Erdheim-Chester disease: characteristics and management.

Erdheim-Chester disease is a rare CD68(+), CD1a(-) non-Langerhans cell histiocytosis with multiorgan involvement. The etiology of Erdheim-Chester disease is unclear; there are no known associated infectious or hereditary genetic abnormalities. However, somatic BRAF mutations have recently been identified in these patients. Historically, the literature regarding the management of Erdheim-Chester disease consisted of case reports and small case series with anecdotal therapeutic responses to agents including, but not limited to, cytotoxic chemotherapy, bone marrow transplantation, cladribine, corticosteroids, IFN-α, the BCR-ABL/KIT inhibitor imatinib mesylate, the IL-1 receptor antagonist anakinra, the TNF-inhibitor infliximab, and recently the BRAF inhibitor vemurafenib. We performed a search of the literature using PubMed with the terms Erdheim Chester disease, without date limitations, including case reports, case series, original articles, and previous review articles. In the absence of large-scale studies, experience-based management prevails. The present review details our approach to the management of patients with Erdheim-Chester disease.
AuthorsJavier Munoz, Filip Janku, Philip R Cohen, Razelle Kurzrock
JournalMayo Clinic proceedings (Mayo Clin Proc) Vol. 89 Issue 7 Pg. 985-96 (Jul 2014) ISSN: 1942-5546 [Electronic] England
PMID24814521 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)
CopyrightCopyright © 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.
Chemical References
  • Bone Density Conservation Agents
  • Immunosuppressive Agents
  • Algorithms
  • Bone Density Conservation Agents (therapeutic use)
  • Bone Marrow Transplantation
  • Combined Modality Therapy
  • Decision Support Techniques
  • Diagnosis, Differential
  • Erdheim-Chester Disease (diagnosis, etiology, physiopathology, therapy)
  • Humans
  • Immunosuppressive Agents (therapeutic use)
  • Prognosis

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