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| ==Rosai-Dorfman disease== | | ==Rosai-Dorfman disease== |
| *Abbreviated ''RDD''. | | *Abbreviated ''RDD''. |
| ===General===
| | *[[AKA]] ''sinus histiocytosis with massive lymphadenopathy'', abbreviated ''SHML''. |
| *[[AKA]] ''sinus histiocytosis with massive lymphadenopathy'', abbreviated ''SHML''.<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> | | {{Main|Rosai-Dorfman disease}} |
| *Super rare.
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| *Prognosis - good.
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| 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>
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| *Fever.
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| *Leukocytosis with neutrophilia.
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| *Polyclonal gamaglobulinemia.
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| ===Microscopic===
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| Features:
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| *Sinus histiocytosis:
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| **Histiocytes - abundant.
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| ***Singular large round nuclei<ref>Bailey, D. 24 August 2010.</ref> ~2x the size of resting lymphocyte.
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| ****Prominent nucleolus - visible with 10x objective.
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| ***Abundant cytoplasm.
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| *Emperipolesis (from ''Greek'': ''em'' = inside, ''peri'' = around, ''polemai'' = wander about<ref>Stedman's Medical Dictionary. 27th Ed.</ref>):
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| **Histiocytes contain other whole cells: neutrophils, lymphocytes, plasma cells.
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| ***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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| ***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>
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| DDx:
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| *Other histiocytosis:
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| **[[Langerhans cell histiocytosis]].
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| **[[Erdheim-Chester disease]].
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| *Infection, e.g. [[rhinoscleroma]] (nasopharynx), [[xanthogranulomatous pyelonephritis]].
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| *Xanthomatous change.
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| ====Images====
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| <gallery>
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| Image:Emperipolesis_-_very_high_mag.jpg | Emperipolesis in SHML (WC)
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| Image:Rosai-Dorfman_disease_-_very_high_mag.jpg | Rosai-Dorfman disease (WC)
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| Image:Rosai-dorfman.jpg | Rosai-Dorfman disease - S100 showing emperipolesis (WC)
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| </gallery>
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| www:
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| *[http://path.upmc.edu/cases/case318.html RDD - case 1 - several images (upmc.edu)].
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| *[http://path.upmc.edu/cases/case338.html RDD - case 2 - several images of breast (upmc.edu)].
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| *[http://path.upmc.edu/cases/case351/micro.html RDD - case 3 - several images (upmc.edu)].
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| *[http://path.upmc.edu/cases/case546.html RDD - case 4 - several images (upmc.edu)].
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| ===IHC===
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| *CD68 +ve.
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| *S100 +ve.
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| **Useful for seeing emperipolesis.
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| *CD1a -ve.
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| **CD1a positive in Langerhans cell histiocytosis.
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| ==Langerhans cell histiocytosis== | | ==Langerhans cell histiocytosis== |