Rosai-dorfman disease

Abstract

Rosai-Dorfman disease (RDD) is a benign non-Langerhans cell histiocytic disorder that usually presents with massive lymphadenopathy and sinus histiocytosis, although at least 40% of patients can have extranodal involvement. Recent studies found NRAS, KRAS, and ARAF mutations in a subset of patients with RDD, but no BRAF-V600E mutations have been reported yet. RDD is often associated with autoimmune or malignant disease, and rare cases of hereditary RDD have been reported as well. In most cases, RDD is self-limited and observation is the standard approach. However, 7-12% of patients die from the disease. Treatment is restricted to patients with life-threatening, multiple-relapsing, or autoimmune-associated disease.