Rosai-Dorfman disease
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| 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]
- 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.
- Singular large round nuclei[4] ~2x the size of resting lymphocyte.
- Histiocytes - abundant.
- Emperipolesis (from Greek: em = inside, peri = around, polemai = wander about[5]):
DDx:
- Other specific histiocytoses:
- Infection, e.g. rhinoscleroma (nasopharynx), xanthogranulomatous pyelonephritis.
- Sinus histiocytosis.
- Xanthomatous change.
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
- RDD - case 1 - several images (upmc.edu).
- RDD - case 2 - several images of breast (upmc.edu).
- RDD - case 3 - several images (upmc.edu).
- RDD - case 4 - several images (upmc.edu).
IHC
Features:[8]
- CD68 +ve.
- S-100 +ve.
- Useful for seeing emperipolesis.
- CD1a -ve.
- CD1a +ve in Langerhans cell histiocytosis.
Images
Rosai-Dorfman disease - S-100 showing emperipolesis (WC/AFIP)
See also
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 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.
- ↑ Bailey, D. 24 August 2010.
- ↑ Stedman's Medical Dictionary. 27th Ed.
- ↑ 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.
- ↑ 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.
- ↑ 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.