Sinus Histiocytosis with Massive Lymphadenopathy
Clinical
- Median age 20 years
- Range from congenital to 74 years
- Rare reports in siblings
- World-wide distribution
- Original reports from Africa
- 90% involve cervical nodes
- May present with extranodal disease
- Most commonly in skin, upper airways, bone
- Associated laboratory abnormalities
- Polyclonal hypergammaglobulinemia frequent
- Elevated sedimentation rate frequent
- Mild anemia
- Spontaneous regression frequent
- Some patients die with progressive disease
