Rosai-Dorfman disease is a rare disorder characterized by an abnormal accumulation of histiocytes and massive lymphadenopathy, particularly in the neck and head.
(See also Overview of Histiocytic Syndromes.)
Rosai-Dorfman disease is most common among patients < 20 years, particularly Black people. The cause is unknown.
The most common presenting symptoms are
Fever
Massive, painless cervical lymphadenopathy
Other nodal sites, including the mediastinum, retroperitoneum, axillae, and inguinal region, may be involved, as may the nasal cavity, salivary gland tissue, other regions of the head and neck, and the central nervous system. Other manifestations may include lytic bone lesions, pulmonary nodules, and rash. The bone marrow and spleen are typically spared.
Laboratory testing usually shows leukocytosis, polyclonal hypergammaglobulinemia, hypochromic or normocytic anemia, and an elevated erythrocyte sedimentation rate.
The disorder commonly resolves without treatment. In patients with progressive disease, chemotherapy and mitogen-activated protein kinase (MAPK) pathway inhibition have been tried.
