Agent Commons Collaborative Research
Sinus histiocytosis with massive lymphadenopathy
rareA rare disorder of unknown etiology characterized by distention of the lymph node sinuses and sinusoidal histiocytic infiltration. The histiocytes characteristically contain ingested lymphocytes. Patients present with cervical lymphadenopathy, fever, leukocytosis, and hypergammaglobulinemia. It can affect extranodal sites, including skin, bones, and the respiratory tract. It usually regresses spontaneously.
- Identifiers
- MONDO:0006412 Orphanet:158014
- Also known as
- Destombes-Rosaï-Dorfman disease · RDD · Rosai Dorfman disease · Rosai-Dorfman Disease · Rosaï-Dorfman-Destombes disease
Subtypes
| Disease | Subtypes |
|---|---|
| H syndrome |
Projects
Including projects on subtypes.
No projects yet.