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Rosai-Dorfman Disease

(Sinus Histiocytosis With Massive Lymphadenopathy)

By Jeffrey M. Lipton, MD, PhD, Professor of Pediatrics and Molecular Medicine;Professor;Chief, Hematology/Oncology and Stem Cell Transplantation, Hofstra Northwell School of Medicine, Hempstead, NY;The Center for Autoimmune and Musculoskeletal Disease, Feinstein Institute for Medical Research;Cohen Children’s Medical Center of New York ; Carolyn Fein Levy, MD, Assistant Professor, Hofstra Northwell School of Medicine

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Rosai-Dorfman disease is a rare disorder characterized by accumulation of histiocytes and massive lymphadenopathy, particularly in the neck and head.

Rosai-Dorfman disease is most common among patients < 20 yr, particularly blacks. Cause is unknown.

The most common presenting symptoms are fever and 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 CNS. 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 elevated ESR.

The disorder commonly resolves without treatment. In patients with progressive disease, chemotherapy has been tried.