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sinus histiocytosis with massive lymphadenopathy - Clinical Profile, Genes & Research | Kisho | Kisho

Rare Disease

sinus histiocytosis with massive lymphadenopathy

MONDO:0006412

Also known as:Destombes-RosaC/-Dorfman diseaseDestombes-Rosaï-Dorfman diseaseRDD

Find Clinical TrialsTrials

Overview

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.

OnsetCONGENITAL

Clinical Trials6 registered · 5 recruiting

Diagnosis

Biomarker and diagnostic research for sinus histiocytosis with massive lymphadenopathy has been reported in the published literature.

Prognosis

Estimated prevalence: <1 in 1,000,000 (VERY_RARE).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

6 clinical trials registered, 5 recruiting. Interventions under study include drug therapy and other interventions. Pipeline includes 2 PHASE2, 1 EARLY_PHASE1. Research is primarily sponsored by academic and government institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT02285582](https://clinicaltrials.gov/study/NCT02285582)

Data assembled from 4 of 12 sources · Last updated Sep 20, 2026, 6:02 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:158014

GARD

Genetic and Rare Diseases Info Center

GARD:7588

Questions for Your Care Team

→Are any of the 6 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

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Research Type	Count	% of Total
Patient case studies	82	56%
Research summaries	45	31%
Disease patterns and progression	8	5%
Laboratory research	5	3%
Other research	2	1%
Testing and diagnosis research	2	1%
Clinical study results	2	1%

sinus histiocytosis with massive lymphadenopathy

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Subtypes

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

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