sinus histiocytosis with massive lymphadenopathy
Findings
No curated finding names sinus histiocytosis with massive lymphadenopathy yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
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.
Definition from the Mondo Disease Ontology (MONDO:0006412), read 2026-09-29. CC BY 4.0.
Features
12 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal circulating immunoglobulin concentrationHPOHP:0010701
- Very frequent (80% to 99% of cases)
- AnemiaHPOHP:0001903
- Very frequent (80% to 99% of cases)
- FeverHPOHP:0001945
- Very frequent (80% to 99% of cases)
- LymphadenopathyHPOHP:0002716
- Very frequent (80% to 99% of cases)
- ErythemaHPOHP:0010783
- Occasional (5% to 29% of cases)
- HeadacheHPOHP:0002315
- Occasional (5% to 29% of cases)
- Osteolysis
Where it sits
- A kind of
- Narrower terms (1)
Other names
6 names
Resolves to: sinus histiocytosis with massive lymphadenopathy
- Also called
- Destombes-Rosaï-Dorfman diseaseRDDRosai Dorfman diseaseRosai-Dorfman DiseaseRosaï-Dorfman-Destombes diseaseSHML