Difference between revisions of "Rosai-Dorfman disease"

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==General==
==General==
*Super rare.
*Super rare.
*Prognosis - good.
*Prognosis - good.<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.

Revision as of 21:11, 1 December 2013

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
Site lymph nodes - see lymph node pathology

Prevalence rare

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.[2]

Clinical findings:[3][2]

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

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

www:

IHC

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 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.