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Rosaï-Dorfman disease

Disease definition

A rare non-Langerhans cell histiocytosis characterized by infiltration of lymph nodes or extranodal tissues by non-malignant histiocytes displaying emperipolesis, a non-destructive phagocytosis of lymphocytes or erythrocytes. Most typical presentation is as a massive cervical lymphadenopathy in adolescents and young adults. Most frequent sites of extranodal disease are skin, soft tissue, bones, paranasal sinuses, orbit, salivary glands, and central nervous system. Symptoms are related to mass effect in the affected organs.

ORPHA:158014

Classification level: Disorder
  • Synonym(s):
    • Destombes-Rosaï-Dorfman disease
    • Rosaï-Dorfman-Destombes disease
    • SHML
    • Sinus histiocytosis with massive lymphadenopathy
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Adolescent, Adult
  • ICD-10: D76.3
  • ICD-11: EK92
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: 7588
  • MedDRA: 10063397

  A summary on this disease is available in Español (2020) Français (2020) Nederlands (2020) Deutsch (2013) Italiano (2013) Russian (2013, pdf) Polski (2013, pdf)

Detailed information

Guidelines

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.