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"
Line 7: Line 7:
!   
!   
!  Histologic features
!  Histologic features
!  EM features
!  [[Electron microscopy|EM]] features
!  CD68
!  CD68
!  S-100
!  S-100
!  CD1a
!  CD1a
!  Image
|-
|-
|  Macrophage
|  Macrophage
|  epithelioid cells, giant cells
|  epithelioid cells, [[giant cells]]
|  -
|  -
|  +
|  +
|  -
|  -
|  -
|  -
|  [http://en.wikipedia.org/wiki/File:Xanthogranulomatous_pyelonephritis_cd68.jpg Macrophages - CD68 (WC)]
|-
|-
|  Erdheim-Chester disease
|  [[Erdheim-Chester disease]]
|  Touton giant cells
|  [[Touton giant cells]]
|  -
|  -
|  +
|  +
|  +/-
|  +/-
|  -
|  -
|  [http://path.upmc.edu/cases/case572.html ECD (upmc.edu)]
|-
|-
|  [[Rosai-Dorfman disease|Rosai-Dorfman]]
|  [[Rosai-Dorfman disease|Rosai-Dorfman]]
Line 32: Line 35:
|  +
|  +
|  -
|  -
|  [http://commons.wikimedia.org/w/index.php?title=File:Emperipolesis_-_very_high_mag.jpg RDD (WC)]
|-
|-
|  Langerhans-histiocytosis
|  [[Langerhans cell histiocytosis]] (LCH)
|  Reniform nuclei,<br>eosinophilic cytoplasm
|  Reniform nuclei,<br>eosinophilic cytoplasm
|  Birbeck granules
|  Birbeck granules
Line 39: Line 43:
|  +
|  +
|  +
|  +
|  [http://commons.wikimedia.org/wiki/File:Langerhans_cell_histiocytosis_-_very_high_mag.jpg LCH (WC)]
|}
|}
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==
{{Main|Langerhans cell histiocytosis}}
===IHC===
Langerin (CD207).<ref name=omim604862>{{OMIM|604862}}</ref>
==Erdheim-Chester disease==
*Abbreviated ''ECD''.
===General===
*Rare.
*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===
Features:
*Bone involvement - classic.
*Usually multiple systems are involved.
Note:
*Does ''not'' usually involve the lymph nodes - like [[Rosai-Dorfman disease]].
===Microscopic===
Features:<ref name=pmid15509691>{{Cite journal  | last1 = Sheu | first1 = SY. | last2 = Wenzel | first2 = RR. | last3 = Kersting | first3 = C. | last4 = Merten | first4 = R. | last5 = Otterbach | first5 = F. | last6 = Schmid | first6 = KW. | title = Erdheim-Chester disease: case report with multisystemic manifestations including testes, thyroid, and lymph nodes, and a review of literature. | journal = J Clin Pathol | volume = 57 | issue = 11 | pages = 1225-8 | month = Nov | year = 2004 | doi = 10.1136/jcp.2004.018481 | PMID = 15509691 }}</ref>
*Foamy histocytes.
**Large cells with gray, bubbly cytoplasm.
*+/-Touton giant cells - very distinctive histiocytes - '''classic'''.
**Nuclei form a ring around the cell periphery.
DDx:
*[[Rosai-Dorfman disease]].
*[[Langerhans cell histiocytosis]].
====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)].
===IHC===
Features:<ref name=pmid17405844>{{Cite journal  | last1 = Haroche | first1 = J. | last2 = Amoura | first2 = Z. | last3 = Touraine | first3 = P. | last4 = Seilhean | first4 = D. | last5 = Graef | first5 = C. | last6 = Birmelé | first6 = B. | last7 = Wechsler | first7 = B. | last8 = Cluzel | first8 = P. | last9 = Grenier | first9 = PA. | title = Bilateral adrenal infiltration in Erdheim-Chester disease. Report of seven cases and literature review. | journal = J Clin Endocrinol Metab | volume = 92 | issue = 6 | pages = 2007-12 | month = Jun | year = 2007 | doi = 10.1210/jc.2006-2018 | PMID = 17405844 }}
</ref>
*CD68 +ve.
*S100 -ve/+ve.
*CD1a -ve.


==See also==
==See also==
Line 48: 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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