Difference between revisions of "Histiocytoses"

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'''Histiocytoses''', singular '''histiocytosis''', are a rare set of conditions affecting tissue macrophages.
'''Histiocytoses''', singular '''histiocytosis''', are a rare set of conditions affecting tissue macrophages.


==Histiocytoses in a table==
==Historical classification of histiocytoses in a table==
Features of histiocytoses:<ref>{{Ref Sternberg4|479}}</ref>  
Features of histiocytoses:<ref>{{Ref Sternberg4|479}}</ref>  
{| class="wikitable" border="1"
{| class="wikitable" border="1"
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!   
!   
!  Histologic features
!  Histologic features
!  [[EM]] features
!  [[Electron microscopy|EM]] features
!  CD68
!  CD68
!  S-100
!  S-100
Line 47: Line 47:
Notes:
Notes:
*''EM'' = [[electron microscopy]].
*''EM'' = [[electron microscopy]].
The classification has evolved considerably, as the entities can overlap.  An overview of a more recent classification is found in the article ''[[classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages]]''.


==Langerhans cell histiocytosis==
==Langerhans cell histiocytosis==
{{Main|Langerhans cell histiocytosis}}
{{Main|Langerhans cell histiocytosis}}
===IHC===
===IHC===
Langerin (CD207).<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/604862 http://www.ncbi.nlm.nih.gov/omim/604862]. Accessed on: 2 February 2011.</ref>
Langerin (CD207).<ref name=omim604862>{{OMIM|604862}}</ref>


==Erdheim-Chester disease==
==Erdheim-Chester disease==
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===General===
===General===
*Rare.
*Rare.
*Non-clonal proliferation of histiocytes.<ref name=pmid12065781>{{Cite journal  | last1 = Al-Quran | first1 = S. | last2 = Reith | first2 = J. | last3 = Bradley | first3 = J. | last4 = Rimsza | first4 = L. | title = Erdheim-Chester disease: case report, PCR-based analysis of clonality, and review of literature. | journal = Mod Pathol | volume = 15 | issue = 6 | pages = 666-72 | month = Jun | year = 2002 | doi = 10.1038/modpathol.3880583 | PMID = 12065781 }}</ref>
*Clonal proliferation of histiocytes; 3 of 5 cases shown monoclonal by Chetritt ''el al''.<ref name=pmid10492045>{{Cite journal  | last1 = Chetritt | first1 = J. | last2 = Paradis | first2 = V. | last3 = Dargere | first3 = D. | last4 = Adle-Biassette | first4 = H. | last5 = Maurage | first5 = CA. | last6 = Mussini | first6 = JM. | last7 = Vital | first7 = A. | last8 = Wechsler | first8 = J. | last9 = Bedossa | first9 = P. | title = Chester-Erdheim disease: a neoplastic disorder. | journal = Hum Pathol | volume = 30 | issue = 9 | pages = 1093-6 | month = Sep | year = 1999 | doi = 10.1016/s0046-8177(99)90228-9 | PMID = 10492045 }}</ref>


===Gross===
===Gross===
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*Bone involvement - classic.
*Bone involvement - classic.
*Usually multiple systems are involved.
*Usually multiple systems are involved.
Note:
*Does ''not'' usually involve the lymph nodes - like [[Rosai-Dorfman disease]].


===Microscopic===
===Microscopic===
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*Foamy histocytes.
*Foamy histocytes.
**Large cells with gray, bubbly cytoplasm.
**Large cells with gray, bubbly cytoplasm.
*+/-Touton giant cells - very distinctive - '''classic'''.
*+/-Touton giant cells - very distinctive histiocytes - '''classic'''.
**Nuclei around cell periphery.
**Nuclei form a ring around the cell periphery.


DDx:
DDx:
Line 75: Line 80:
*[[Langerhans cell histiocytosis]].  
*[[Langerhans cell histiocytosis]].  


Images:
====Images====
<gallery>
Image: Maladie de Chester-Erdheim.png |EHC. (WC)
</gallery>
www:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770491/figure/f1/ ECD (nih.gov)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770491/figure/f1/ ECD (nih.gov)].


Line 89: Line 98:
*[[Neuropathology]].
*[[Neuropathology]].
*[[Hemophagocytic syndrome]].
*[[Hemophagocytic syndrome]].
*[[Histiocytic sarcoma]].


==References==
==References==
{{reflist|1}}
{{reflist|2}}


[[Category:Weird stuff]]
[[Category:Weird stuff]]

Latest revision as of 18:55, 23 July 2019

Histiocytoses, singular histiocytosis, are a rare set of conditions affecting tissue macrophages.

Historical classification of histiocytoses in a table

Features of histiocytoses:[1]

Histologic features EM features CD68 S-100 CD1a Image
Macrophage epithelioid cells, giant cells - + - - Macrophages - CD68 (WC)
Erdheim-Chester disease Touton giant cells - + +/- - ECD (upmc.edu)
Rosai-Dorfman Emperipolesis - + + - RDD (WC)
Langerhans cell histiocytosis (LCH) Reniform nuclei,
eosinophilic cytoplasm 		Birbeck granules + + + LCH (WC)

Notes:

The classification has evolved considerably, as the entities can overlap. An overview of a more recent classification is found in the article classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages.

Langerhans cell histiocytosis

Main article: Langerhans cell histiocytosis

IHC

Langerin (CD207).[2]

Erdheim-Chester disease

  • Abbreviated ECD.

General

  • Rare.
  • Clonal proliferation of histiocytes; 3 of 5 cases shown monoclonal by Chetritt el al.[3]

Gross

Features:

  • Bone involvement - classic.
  • Usually multiple systems are involved.

Note:

Microscopic

Features:[4]

  • Foamy histocytes.
    • Large cells with gray, bubbly cytoplasm.
  • +/-Touton giant cells - very distinctive histiocytes - classic.
    • Nuclei form a ring around the cell periphery.

DDx:

Images

  • EHC. (WC)

www:

IHC

Features:[5]

  • CD68 +ve.
  • S100 -ve/+ve.
  • CD1a -ve.

See also

References

  1. ↑ Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 479. ISBN 978-0781740517.
  2. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 604862
  3. ↑ Chetritt, J.; Paradis, V.; Dargere, D.; Adle-Biassette, H.; Maurage, CA.; Mussini, JM.; Vital, A.; Wechsler, J. et al. (Sep 1999). "Chester-Erdheim disease: a neoplastic disorder.". Hum Pathol 30 (9): 1093-6. doi:10.1016/s0046-8177(99)90228-9. PMID 10492045.
  4. ↑ Sheu, SY.; Wenzel, RR.; Kersting, C.; Merten, R.; Otterbach, F.; Schmid, KW. (Nov 2004). "Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature.". J Clin Pathol 57 (11): 1225-8. doi:10.1136/jcp.2004.018481. PMID 15509691.
  5. ↑ Haroche, J.; Amoura, Z.; Touraine, P.; Seilhean, D.; Graef, C.; Birmelé, B.; Wechsler, B.; Cluzel, P. et al. (Jun 2007). "Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review.". J Clin Endocrinol Metab 92 (6): 2007-12. doi:10.1210/jc.2006-2018. PMID 17405844.
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