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Rosai-Dorfman Disease

(Sinus Histiocytosis With Massive Lymphadenopathy)

By

Jeffrey M. Lipton

, MD, PhD, Zucker School of Medicine at Hofstra/Northwell;


Carolyn Fein Levy

, MD, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell

Last full review/revision Dec 2021
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Rosai-Dorfman disease is a rare disorder characterized by an abnormal 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

  • 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.

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