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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 | *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) | ||
</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)]. | ||
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==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. | ||
* | *S-100 +ve. | ||
**Useful for seeing emperipolesis. | **Useful for seeing emperipolesis. | ||
*CD1a -ve. | *CD1a -ve. | ||
**CD1a | **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== | ||