Erdheim-Chester disease laboratory findings

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Erdheim-Chester disease Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Laboratory Findings

Histologically, ECD differs from Langerhans cell histiocytosis (LCH) in a number of ways. Unlike LCH, ECD does not stain positive for S-100 or CD 1a, and electron microscopy of cell cytoplasm does not disclose Birbeck granules.[1] Tissue samples show xanthomatous or xanthogranulomatous infiltration by lipid-laden or foamy histiocytes, and are usually surrounded by fibrosis. Bone biopsy is said to offer the greatest likelihood of reaching a diagnosis.

ECD is not a common cause of exophthalmos but can be diagnosed by biopsy. However, like all biopsies, this may be inconclusive.[2]

References

  1. Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D; et al. (1996). "Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases". Medicine (Baltimore). 75 (3): 157–69. PMID 8965684.
  2. "Erdheim Chester Disease - M. D. Anderson Cancer Center". Retrieved 2007-08-26.