sinus histiocytosis with massive lymphadenopathy https://github.com/monarch-initiative/mondo/issues/4236 https://www.malacards.org/card/histiocytosis_lymphadenopathy_plus_syndrome SHML Rosai-Dorfman Disease RDD SCTID:34287003 NORD:1676 MONDO:0006412 Orphanet:158014 NANDO:2200039 icd11.foundation:1908538383 ICD9:277.89 sinus histiocytosis with massive lymphadenopathy NCIT:C36075 Destombes-Rosaï-Dorfman disease Rosaï-Dorfman-Destombes disease Destombes-RosaC/-Dorfman disease MEDGEN:9266 RosaC/-Dorfman-Destombes disease ONCOTREE:RDD UMLS:C0019625 GARD:0007588 MESH:D015618 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. Rosai-Dorfman disease non-Langerhans cell histiocytosis