Rosai–Dorfman disease

The Rosai - Dorfman syndrome (Alternative name: sinus histiocytosis with massive lymphadenopathy, English: Rosai - Dorfman disease) is a rare disease from the group of histiocytosis. The disease, which was described in 1969 by the American pathologist Juan Rosai and Ronald F. Dorfman for the first time, is characterized by a proliferation of histiocytes which accumulate mainly in the lymph nodes. The cause is unknown.

Pathology

It is shown a pronounced two-sided increase in the cervical lymph node, which is mainly due to the widening of the sine of the lymph nodes. These sinuses are filled with activated histiocytes which envelop intact erythrocytes and lymphocytes, which is called Emperipolese. At the same time an increase in plasma cells, lymphocytes and neutrophils occurs in the sinus. Other locations are possible: as foci in the skin, brain skin, the upper respiratory tract, salivary glands, and bone marrow can occur. The cause is as yet unclear, it is believed that viral infections may play a role.

Symptoms

In Europe, the Rosai - Dorfman syndrome is very rare and primarily concerns Adolescents aged 15 to 20 years, people around the world black skin are more commonly affected. In addition to pronounced swelling of lymph nodes ( lymphadenopathy) General symptoms such as fever and weight loss may occur .. In about half the cases, there are foci of disease outside of the lymph nodes (skin, upper respiratory tract, salivary glands ), which then show up with specific symptoms for the Lokalisationsort. The disease is self-limited, laboratory tests, there are general signs of inflammation.