Sinus histiocytosis with massive lymphadenopathy
Sinus histiocytosis with massive lymphadenopathy
Definition
A 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.
Also known as Destombes-RosaC/-Dorfman disease, Destombes-Rosaï-Dorfman disease, RDD, RosaC/-Dorfman-Destombes disease, Rosai-Dorfman Disease, Rosaï-Dorfman-Destombes disease, SHML, sinus histiocytosis with massive lymphadenopathy — per MONDO
Also identified as
- ICD9 277.89 per MONDO
- MESH D015618 per MONDO
- NCIT C36075 per MONDO
- Orphanet 158014 per MONDO
- SCTID 34287003 per MONDO
- UMLS C0019625 per MONDO
Affected Organs
| Organ | Involvement | Source |
|---|---|---|
| Hematopoietic system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |
| Lymphoid system | Disease Has Associated Anatomic Site | NCIT · CC BY 4.0 |