(Rosai-Dorfman syndrome, sinus histiocytosis with massive lymphadenopathy)
The Rosai-Dorfman disease is a rare disorder that is characterized by an accumulation of histiocytes and massive lymphadenopathy, especially in the neck and head.
Particularly in dark-skinned patients <20 years, Rosai-Dorfman disease occurs. The cause is unknown.
The Rosai-Dorfman disease is a rare disorder that is characterized by an accumulation of histiocytes and massive lymphadenopathy, especially in the neck and head. Particularly in dark-skinned patients <20 years, Rosai-Dorfman disease occurs. The cause is unknown. The most common symptoms are fever and a massive, painless, cervical lymphadenopathy. Other lymph node regions, including the mediastinum, retroperitoneum, armpits and groin may be affected, as well as nasal cavity, salivary glands, other areas of the head and neck and the central nervous system. The clinical presentation may include bone lesions, pulmonary node and exanthema. Spared the bone marrow and the spleen usually remain. The laboratory test results usually show a leukocytosis, a polyclonal hypergammaglobulinemia, hypochromic or normocytic anemia and an elevated ESR. Often without treatment, the disease heals. There is no unique treatment option; Chemotherapy has been tried.