(See also Overview of Histiocytic Syndromes Overview of Histiocytic Disorders The histiocytic disorders are clinically heterogeneous disorders that result from an abnormal proliferation of histiocytes that are either Dendritic cells (antigen-presenting cells) Monocyte-macrophages... read more .)
Rosai-Dorfman disease is most common among patients < 20 years, particularly Black people. The cause is unknown.
The most common presenting symptoms are
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.