Difference between revisions of "Rosai-Dorfman disease"

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'''Rosai-Dorfman disease''', also known as '''sinus histiocytosis with massive lymphadenopathy''', is a rare [[lymph node pathology]].
'''Rosai-Dorfman disease''', abbreviated '''RDD''', is a rare [[lymph node pathology]].
 
It is also known as '''sinus histiocytosis with massive lymphadenopathy''',<ref name=pmid17183839>{{cite journal |author=Agarwal A, Pathak S, Gujral S |title=Sinus histiocytosis with massive lymphadenopathy--a review of seven cases |journal=Indian J Pathol Microbiol |volume=49 |issue=4 |pages=509–15 |year=2006 |month=October |pmid=17183839 |doi= |url=}}</ref> abbreviated '''SHML'''.
 
==General==
*Super rare.
*Prognosis - good.
 
Clinical findings:<ref name=pmid19668942>{{Cite journal  | last1 = Landim | first1 = FM. | last2 = Rios | first2 = Hde O. | last3 = Costa | first3 = CO. | last4 = Feitosa | first4 = RG. | last5 = Rocha Filho | first5 = FD. | last6 = Costa | first6 = AA. | title = Cutaneous Rosai-Dorfman disease. | journal = An Bras Dermatol | volume = 84 | issue = 3 | pages = 275-8 | month = Jul | year = 2009 | doi =  | PMID = 19668942 }}</ref>
*Fever.
*Leukocytosis with neutrophilia.
*Polyclonal gamaglobulinemia.
 
==Microscopic==
Features:
*Sinus histiocytosis:
**Histiocytes - abundant.
***Singular large round nuclei<ref>Bailey, D. 24 August 2010.</ref> ~2x the size of resting lymphocyte.
****Prominent nucleolus - visible with 10x objective.
***Abundant cytoplasm.
*Emperipolesis (from ''Greek'': ''em'' = inside, ''peri'' = around, ''polemai'' = wander about<ref>Stedman's Medical Dictionary. 27th Ed.</ref>):
**Histiocytes contain other whole cells: neutrophils, lymphocytes, plasma cells.
***The "eaten" cell is within a vacuole;<ref>{{cite journal |author=Viswanathan P, Raghunathan K, Majhi U, Pandit RV, Shanthi R, Rajkumar T|title=Emperipolesis : an electron microscopic characteristic in RDD (Rosai-Dorfaman disease) : a case report |volume= |issue=1|pages=14-6 |year=1997 |month= |pmid= |doi= |url=http://www.ijmpo.org/article.asp?issn=0971-5851;year=1997;volume=18;issue=1;spage=14;epage=16;aulast=Viswanathan;type=0}}</ref> thus, it should have a clear halo around it.
***Thought to be related to ''peripolesis''; the attachment of a cell to another.<ref name=pmid1577151>{{cite journal |author=Lyons DJ, Gautam A, Clark J, ''et al.'' |title=Lymphocyte macrophage interactions: peripolesis of human alveolar macrophages |journal=Eur. Respir. J. |volume=5 |issue=1 |pages=59–66 |year=1992 |month=January |pmid=1577151 |doi= |url=}}</ref>
 
DDx:
*Other histiocytosis:
**[[Langerhans cell histiocytosis]].
**[[Erdheim-Chester disease]].
*Infection, e.g. [[rhinoscleroma]] (nasopharynx), [[xanthogranulomatous pyelonephritis]].
*Xanthomatous change.
 
===Images===
<gallery>
Image:Emperipolesis_-_very_high_mag.jpg | Emperipolesis in SHML (WC)
Image:Rosai-Dorfman_disease_-_very_high_mag.jpg | Rosai-Dorfman disease (WC)
Image:Rosai-dorfman.jpg | Rosai-Dorfman disease - S100 showing emperipolesis (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case318.html RDD - case 1 - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case338.html RDD - case 2 - several images of breast (upmc.edu)].
*[http://path.upmc.edu/cases/case351/micro.html RDD - case 3 - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case546.html RDD - case 4 - several images (upmc.edu)].
 
==IHC==
*CD68 +ve.
*S100 +ve.
**Useful for seeing emperipolesis.
*CD1a -ve.
**CD1a positive in Langerhans cell histiocytosis.


==See also==
==See also==

Revision as of 00:52, 1 December 2013

Rosai-Dorfman disease, abbreviated RDD, is a rare lymph node pathology.

It is also known as sinus histiocytosis with massive lymphadenopathy,[1] abbreviated SHML.

General

  • Super rare.
  • Prognosis - good.

Clinical findings:[2]

  • Fever.
  • Leukocytosis with neutrophilia.
  • Polyclonal gamaglobulinemia.

Microscopic

Features:

  • Sinus histiocytosis:
    • Histiocytes - abundant.
      • Singular large round nuclei[3] ~2x the size of resting lymphocyte.
        • Prominent nucleolus - visible with 10x objective.
      • Abundant cytoplasm.
  • Emperipolesis (from Greek: em = inside, peri = around, polemai = wander about[4]):
    • Histiocytes contain other whole cells: neutrophils, lymphocytes, plasma cells.
      • The "eaten" cell is within a vacuole;[5] thus, it should have a clear halo around it.
      • Thought to be related to peripolesis; the attachment of a cell to another.[6]

DDx:

Images

  • Emperipolesis in SHML (WC)

  • Rosai-Dorfman disease (WC)

  • Rosai-Dorfman disease - S100 showing emperipolesis (WC)

www:

IHC

  • CD68 +ve.
  • S100 +ve.
    • Useful for seeing emperipolesis.
  • CD1a -ve.
    • CD1a positive in Langerhans cell histiocytosis.

See also

  1. ↑ Agarwal A, Pathak S, Gujral S (October 2006). "Sinus histiocytosis with massive lymphadenopathy--a review of seven cases". Indian J Pathol Microbiol 49 (4): 509–15. PMID 17183839.
  2. ↑ Landim, FM.; Rios, Hde O.; Costa, CO.; Feitosa, RG.; Rocha Filho, FD.; Costa, AA. (Jul 2009). "Cutaneous Rosai-Dorfman disease.". An Bras Dermatol 84 (3): 275-8. PMID 19668942.
  3. ↑ Bailey, D. 24 August 2010.
  4. ↑ Stedman's Medical Dictionary. 27th Ed.
  5. ↑ Viswanathan P, Raghunathan K, Majhi U, Pandit RV, Shanthi R, Rajkumar T (1997). Emperipolesis : an electron microscopic characteristic in RDD (Rosai-Dorfaman disease) : a case report. pp. 14-6. http://www.ijmpo.org/article.asp?issn=0971-5851;year=1997;volume=18;issue=1;spage=14;epage=16;aulast=Viswanathan;type=0.
  6. ↑ Lyons DJ, Gautam A, Clark J, et al. (January 1992). "Lymphocyte macrophage interactions: peripolesis of human alveolar macrophages". Eur. Respir. J. 5 (1): 59–66. PMID 1577151.
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