Rosai-Dorfman disease

Rosai-Dorfman disease
Micrograph of a lymph node involved by Rosai-Dorfman disease showing emperipolesis. H&E stain.
Classification and external resources
Specialty	hematology
ICD-10	D76.3 (ILDS D76.360)
DiseasesDB	31419
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Rosai–Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare disorder of unknown cause that is characterized by abundant histiocytes in the lymph nodes throughout the body.^,

Lymphadenopathy of the neck is the most common place of histiocyte accumulation, although accumulation outside of lymph nodes may occur, as well. The most common sites of accumulation outside of the lymph nodes are skin, upper respiratory tract, and the sinuses. The disease can be also diagnosed in thyroid gland.

The symptoms of this disease vary with the site of accumulation similar to other regional tumors. For instance, accumulation in closed spaces such as the cranium can lead to poor outcomes compared to growth in the dermis of an extremity where surgical excision is possible.

The disease is usually non-progressive and self-limited, but patients can manifest autoimmune hemolytic anemia. It can rarely present with massive hemoptysis. Extra nodal sites such as skin, mucosae, spinal cord and pancreas may be involved.

In 1969, two pathologists, Juan Rosai and Ronald Dorfman, reported a distinct histiocytic disorder in several patients with massive enlargement of the lymph nodes, as well as other symptoms. They named this condition sinus histiocytosis with massive lymphadenopathy, and the name has since come to be known as Rosai-Dorfman disease.

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