Syndrome
Rosai-Dorfman Disease
Synonyms: Sinus histiocytosis with massive lymphadenopathy, SHML
Benign histiocytic disorder
Quick Facts
Behaviour
Not set
Category
Syndrome
Synonyms
- Sinus histiocytosis with massive lymphadenopathy
- SHML
Category
Syndrome
Gender
M = F
Epidemiology
- Benign histiocytic disorder
- Peak incidence in 1st-2nd decades (juvenile form) or older adults
- African ancestry predominance
- Can involve Bone and soft tissues
Clinical Features
- Massive lymphadenopathy
- Fever, weight loss
- Bone pain if skeletal involvement
Location
- Lymph nodes (mediastinal, cervical)
- Bone and soft tissues less common but can occur
Imaging
- CT: massive mediastinal/cervical lymphadenopathy
- MRI: characterises extent
Pathology
- Sheets of histiocytes with phagocytosed material
- S100+, CD68+
- Benign course
Genetics
- Sporadic or familial
- SLC29A3 mutations in some
Treatment
- Observation for asymptomatic disease
- Chemotherapy or radiotherapy for symptomatic disease
Prognosis
- Generally excellent - self-limited disease
- 50% spontaneous regression
- Others require treatment
Key Points
- Benign histiocytic disorder
- Often self-limited
- Resembles malignancy clinically but Benign
Workup - Blood Tests
FBC, U&E, LFTs - baseline
Workup - Local Imaging
- CT chest/abdomen/pelvis - assess extent
- MRI if soft tissue/Bone involvement
Workup - Biopsy
- Confirm diagnosis via excision or biopsy
- Histology and immunophenotype diagnostic
Workup - Staging
Assess systemic involvement
Medical disclaimer
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