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]

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

www:

IHC

Features:[8]

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.