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Home > West Medicine > Internal Medicine > Hematology
D76.303

Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease)

血液科

Common symptoms

组织细胞增生、免疫性溶血、淋巴结肿大、血沉增快

The pathology of disease

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare benign idiopathic histi-proliferative disorder. It most often presents with painless massive bilateral cervical lymphadenopathy, fever, leukocytosis, and polyclonal hypergammaglobulinemia; it is usually self-limited but may recur or involve extranodal sites (skin, upper respiratory tract, orbit).

Common Symptoms

Painless massive cervical lymphadenopathy; fever; leukocytosis; hypergammaglobulinemia; fatigue

Causes and Pathogenesis

Idiopathic non-neoplastic histi proliferation with emperipolesis; may be associated with EBV/CMV/HHV-6; benign course.

Treatment

Observation; corticosteroids/surgery/radiotherapy for symptomatic extranodal disease

Preventive Measures

Usually self-resolving; follow-up for recurrence/extranodal involvement.

Department to Visit

It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.