sinus histiocytosis with massive lymphadenopathy
http://purl.obolibrary.org/obo/MONDO_0006412
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. [ NCIT:P378 ]
Term info
- EFO:1000528 (MONDO:equivalentTo)
- ONCOTREE:RDD (MONDO:equivalentTo)
- Orphanet:158014 (MONDO:equivalentTo)
- MESH:D015618 (MONDO:equivalentTo)
- NCIT:C36075 (ONCOTREE:RDD)
- GARD:0007588 (MONDO:shared-umls-xref)
- MedDRA:10063397 (Orphanet:158014)
- SCTID:34287003 (MONDO:equivalentTo)
- ICD9:277.89 (MONDO:relatedTo)
ordo_disease
https://github.com/monarch-initiative/mondo/issues/4236
http://identifiers.org/meddra/10063397
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.
http://purl.obolibrary.org/obo/NCIT_C36075, http://identifiers.org/snomedct/34287003, http://identifiers.org/mesh/D015618, http://purl.obolibrary.org/obo/Orphanet_158014
Destombes-RosaC/-Dorfman disease, SHML, sinus histiocytosis with massive lymphadenopathy, RDD, Rosai-Dorfman disease, Destombes-Rosaï-Dorfman disease, RosaC/-Dorfman-Destombes disease, Rosaï-Dorfman-Destombes disease
MONDO:0006412