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Rosai-Dorfman Disease

(Sinus Histiocytosis With Massive Lymphadenopathy)

By

Jeffrey M. Lipton

, MD, PhD, Hofstra Northwell School of Medicine, Hempstead, NY;


Carolyn Fein Levy

, MD, Hofstra Northwell School of Medicine

Last full review/revision Jul 2019| Content last modified Jul 2019
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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 years, particularly blacks. The 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 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 elevated erythrocyte sedimentation rate.

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

Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
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