Erdheim-Chester disease laboratory findings: Difference between revisions
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Histologically, ECD differs from Langerhans cell histiocytosis (LCH) in a number of ways. Unlike LCH, ECD does not stain positive for [[S-100 protein|S-100]] or [[CD 1a]], and electron microscopy of cell cytoplasm does not disclose [[Birbeck granules]].<ref name="pmid8965684">{{cite journal |author=Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, ''et al'' |title=Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases |journal=Medicine (Baltimore) |volume=75 |issue=3 |pages=157-69 |year=1996 |pmid=8965684 |doi=}}</ref> 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. | Histologically, ECD differs from Langerhans cell histiocytosis (LCH) in a number of ways. Unlike LCH, ECD does not stain positive for [[S-100 protein|S-100]] or [[CD 1a]], and electron microscopy of cell cytoplasm does not disclose [[Birbeck granules]].<ref name="pmid8965684">{{cite journal |author=Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, ''et al'' |title=Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases |journal=Medicine (Baltimore) |volume=75 |issue=3 |pages=157-69 |year=1996 |pmid=8965684 |doi=}}</ref> 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.<ref name="Mdanderson"> {{cite web |url=http://www.mdanderson.org/diseases/eyecancer/display.cfm?id=5423a04c-459e-4e12-a737309ef2e1e299&method=displayfull |title=Erdheim Chester Disease - M. D. Anderson Cancer Center |accessdate=2007-08-26 |format= |work=}}</ref> | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Disease]] | |||
[[Category:Rare diseases]] | |||
[[Category:Immune system disorders]] | |||
[[Category:Skeletal disorders]] | |||
[[Category:Syndromes]] | |||
[[Category:Hematology]] | |||
Latest revision as of 14:10, 21 September 2012
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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
- ↑ 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.
- ↑ "Erdheim Chester Disease - M. D. Anderson Cancer Center". Retrieved 2007-08-26.