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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

database cross reference
  • ONCOTREE:RDD (MONDO:equivalentTo)
  • GARD:0007588 (MONDO:shared-umls-xref)
  • NCIT:C36075 (ONCOTREE:RDD)
  • SCTID:34287003 (MONDO:equivalentTo)
  • MESH:D015618 (MONDO:equivalentTo)
  • Orphanet:158014 (MONDO:equivalentTo)
  • EFO:1000528 (MONDO:equivalentTo)
  • ICD9:277.89 (MONDO:relatedTo)
  • MedDRA:10063397 (Orphanet:158014)
Subsets

ordo_disease

abbreviation
SHML [ Orphanet:158014 ]

abbreviation
RDD [ GARD:0007588 ]

IAO 0000233

https://github.com/monarch-initiative/mondo/issues/4236

closeMatch

http://identifiers.org/meddra/10063397

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.

exactMatch

http://purl.obolibrary.org/obo/NCIT_C36075, http://identifiers.org/snomedct/34287003, http://identifiers.org/mesh/D015618, http://purl.obolibrary.org/obo/Orphanet_158014

has exact synonym

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

id

MONDO:0006412

Term relations