Difference between revisions of "Rosai-Dorfman disease"

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{{ Infobox diagnosis  
{{ Infobox diagnosis  
| Name = {{PAGENAME}}
| Name = {{PAGENAME}}
| Image = Emperipolesis_-_very_high_mag.jpg
| Image = Emperipolesis_-_very_high_mag.jpg  
| Width =  
| Width =  
| Caption =  
| Caption = Rosai-Dorfman disease. H&E stain.
| Micro =  
| Micro = sinus histiocytosis with histiocytes have a singular large round nucleus (~2x the size of a lymphocyte) with a prominent nucleolus (visible with 10x objective); emperipolesis
| Subtypes =  
| Subtypes =  
| LMDDx =  
| LMDDx = other specific [[histiocytoses]] ([[Langerhans cell histiocytosis]], [[Erdheim-Chester disease]]), specific infections ([[rhinoscleroma]], [[xanthogranulomatous pyelonephritis]]), [[sinus histiocytosis]]
| Stains =  
| Stains =  
| IHC =  
| IHC = CD68 +ve, S-100 +ve, CD1a -ve
| EM =  
| EM =  
| Molecular =  
| Molecular =  
| IF =  
| IF =  
| Gross =  
| Gross = nodules (skin)
| Grossing =  
| Grossing =  
| Site =  
| Site = [[skin]], [[lymph node]]s - see ''[[lymph node pathology]]''
| Assdx =  
| Assdx =  
| Syndromes =  
| Syndromes =  
| Clinicalhx =  
| Clinicalhx =  
| Signs =  
| Signs = fever, lymphadenopathy
| Symptoms =  
| Symptoms =  
| Prevalence = rare
| Prevalence = rare
| Bloodwork =  
| Bloodwork = leukocytosis with neurophilia
| Rads =  
| Rads =  
| Endoscopy =  
| Endoscopy =  
| Prognosis =  
| Prognosis = usually self-limited, benign
| Other =  
| Other =  
| ClinDDx = }}
| ClinDDx = lymphoma, infections with lymphadenopathy }}
'''Rosai-Dorfman disease''', abbreviated '''RDD''', is a rare [[lymph node pathology]].
'''Rosai-Dorfman disease''', abbreviated '''RDD''', is a rare [[lymph node pathology]].


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==General==
==General==
*Super rare.
*Super rare.
*Prognosis - good.
*Prognosis - good, usually self-limited.<ref name=pmid23739703/>


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>
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><ref name=pmid23739703>{{Cite journal  | last1 = Leal | first1 = PA. | last2 = Adriano | first2 = AL. | last3 = Breckenfeld | first3 = MP. | last4 = Costa | first4 = IS. | last5 = de Sousa | first5 = AR. | last6 = Gonçalves | first6 = Hde S. | title = Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report. | journal = An Bras Dermatol | volume = 88 | issue = 2 | pages = 256-9 | month =  | year =  | doi = 10.1590/S0365-05962013000200014 | PMID = 23739703 }}</ref>
*Fever.
*Fever.
*Leukocytosis with neutrophilia.
*Leukocytosis with neutrophilia.
*Polyclonal gammaglobulinemia.
*Polyclonal gammaglobulinemia.
==Gross==
*Skin: nodules.<ref name=pmid23739703/>
Note:
*Reported at many sites.


==Microscopic==
==Microscopic==
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****Prominent nucleolus - visible with 10x objective.
****Prominent nucleolus - visible with 10x objective.
***Abundant cytoplasm.
***Abundant cytoplasm.
*Emperipolesis (from ''Greek'': ''em'' = inside, ''peri'' = around, ''polemai'' = wander about<ref>Stedman's Medical Dictionary. 27th Ed.</ref>):
*[[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.
**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.
***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.
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DDx:
DDx:
*Other histiocytosis:
*Other specific [[histiocytoses]]:
**[[Langerhans cell histiocytosis]].
**[[Langerhans cell histiocytosis]].
**[[Erdheim-Chester disease]].
**[[Erdheim-Chester disease]].
*Infection, e.g. [[rhinoscleroma]] (nasopharynx), [[xanthogranulomatous pyelonephritis]].
*Infection, e.g. [[rhinoscleroma]] (nasopharynx), [[xanthogranulomatous pyelonephritis]].
*[[Sinus histiocytosis]].
*Xanthomatous change.
*Xanthomatous change.


===Images===
===Images===
====Case 1====
<gallery>
<gallery>
Image:Emperipolesis_-_very_high_mag.jpg | Emperipolesis in SHML (WC)
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_disease_-_very_high_mag.jpg | Rosai-Dorfman disease (WC)
Image:Rosai-dorfman.jpg | Rosai-Dorfman disease - S100 showing emperipolesis (WC)
</gallery>
</gallery>
www:
====Case 2====
<gallery>
Image: Rosai-Dorfman disease of parotid gland -- low mag.jpg | RDD - low mag.
Image: Rosai-Dorfman disease of parotid gland -- intermed mag.jpg | RDD - intermed. mag.
Image: Rosai-Dorfman disease of parotid gland -- high mag.jpg | RDD - high mag.
Image: Rosai-Dorfman disease of parotid gland - alt -- high mag.jpg | RDD - high mag.
Image: Rosai-Dorfman disease of parotid gland -- very high mag.jpg | RDD - very high mag.
</gallery>
====www====
*[http://path.upmc.edu/cases/case318.html RDD - case 1 - several images (upmc.edu)].
*[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/case338.html RDD - case 2 - several images of breast (upmc.edu)].
Line 72: Line 87:


==IHC==
==IHC==
Features:<ref name=pmid18504582>{{Cite journal  | last1 = Hartmann | first1 = S. | last2 = Kriener | first2 = S. | last3 = Hansmann | first3 = ML. | title = [Diagnostic spectrum of reactive lymph node changes]. | journal = Pathologe | volume = 29 | issue = 4 | pages = 253-63 | month = Jul | year = 2008 | doi = 10.1007/s00292-008-1003-5 | PMID = 18504582 }}</ref>
*CD68 +ve.
*CD68 +ve.
*S100 +ve.
*S-100 +ve.
**Useful for seeing emperipolesis.
**Useful for seeing emperipolesis.
*CD1a -ve.
*CD1a -ve.
**CD1a positive in Langerhans cell histiocytosis.
**CD1a +ve in [[Langerhans cell histiocytosis]].
 
===Images===
<gallery>
Image:Rosai-dorfman.jpg | Rosai-Dorfman disease - S-100 showing emperipolesis (WC/AFIP)
</gallery>


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

Latest revision as of 15:25, 2 June 2020

Rosai-Dorfman disease
Diagnosis in short

Rosai-Dorfman disease. H&E stain.
LM sinus histiocytosis with histiocytes have a singular large round nucleus (~2x the size of a lymphocyte) with a prominent nucleolus (visible with 10x objective); emperipolesis
LM DDx other specific histiocytoses (Langerhans cell histiocytosis, Erdheim-Chester disease), specific infections (rhinoscleroma, xanthogranulomatous pyelonephritis), sinus histiocytosis
IHC CD68 +ve, S-100 +ve, CD1a -ve
Gross nodules (skin)
Site skin, lymph nodes - see lymph node pathology
Signs fever, lymphadenopathy
Prevalence rare
Blood work leukocytosis with neurophilia
Prognosis usually self-limited, benign
Clin. DDx lymphoma, infections with lymphadenopathy

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, usually self-limited.[2]

Clinical findings:[3][2]

  • Fever.
  • Leukocytosis with neutrophilia.
  • Polyclonal gammaglobulinemia.

Gross

  • Skin: nodules.[2]

Note:

  • Reported at many sites.

Microscopic

Features:

  • Sinus histiocytosis:
    • Histiocytes - abundant.
      • Singular large round nuclei[4] ~2x the size of resting lymphocyte.
        • Prominent nucleolus - visible with 10x objective.
      • Abundant cytoplasm.
  • Emperipolesis (from Greek: em = inside, peri = around, polemai = wander about[5]):
    • Histiocytes contain other whole cells: neutrophils, lymphocytes, plasma cells.
      • The "eaten" cell is within a vacuole;[6] thus, it should have a clear halo around it.
      • Thought to be related to peripolesis; the attachment of a cell to another.[7]

DDx:

Images

Case 1

  • Emperipolesis in SHML (WC)

  • Rosai-Dorfman disease (WC)

Case 2

  • RDD - low mag.

  • RDD - intermed. mag.

  • RDD - high mag.

  • RDD - high mag.

  • RDD - very high mag.

www

IHC

Features:[8]

Images

  • Rosai-Dorfman disease - S-100 showing emperipolesis (WC/AFIP)

See also

References

  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. ↑ 2.0 2.1 2.2 Leal, PA.; Adriano, AL.; Breckenfeld, MP.; Costa, IS.; de Sousa, AR.; Gonçalves, Hde S.. "Rosai-Dorfman disease presenting with extensive cutaneous manifestation - case report.". An Bras Dermatol 88 (2): 256-9. doi:10.1590/S0365-05962013000200014. PMID 23739703.
  3. ↑ 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.
  4. ↑ Bailey, D. 24 August 2010.
  5. ↑ Stedman's Medical Dictionary. 27th Ed.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ Hartmann, S.; Kriener, S.; Hansmann, ML. (Jul 2008). "[Diagnostic spectrum of reactive lymph node changes].". Pathologe 29 (4): 253-63. doi:10.1007/s00292-008-1003-5. PMID 18504582.
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