Rosaï-Dorfman disease

Orpha code: 158014OMIM code:

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.

Disease data
Classification

Disease

Synonyms
Destombes-Rosaï-Dorfman disease
Choroba Destombesa, Rosaï i Dorfmana
choroba Rosaï, Dorfmana i Destombesa
Histiocytoza zatokowa z masywną limfadenopatią
SHML
Rosaï-Dorfman-Destombes disease
SHML
Sinus histiocytosis with massive lymphadenopathy
ORPHA code
158014
OMIM code
-
ICD10 code
D76.3
ICD11 code
EK92

No additional description.

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