Merck Manual

Please confirm that you are a health care professional

honeypot link

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 Jul 2019| Content last modified Jul 2019
Click here for Patient Education

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
Professionals also read
Test your knowledge
Langerhans Cell Histiocytosis
A 10-year-old girl is brought to the clinic by her father because she has had pain in her right upper leg for the past 3 months. On physical examination, the patient is unable to bear weight on the limb. Edema of the upper leg and tenderness to palpation are noted. X-rays show 2 bone lesions with sharp margins and a punched-out appearance. Which of the following is the most appropriate definitive step in diagnosis?
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
Download the Manuals App iOS ANDROID
 

Also of Interest

 
TOP