Destombes Rosai Dorfman Syndrome (DRDS), also known as Rosai-Dorfman disease or Sinus Histiocytosis with Massive Lymphadenopathy (SHML), is a rare non-cancerous disorder characterized by the overproduction and accumulation of immune cells, known as histiocytes, throughout the body.
The condition typically presents with painless, enlarged lymph nodes, particularly in the neck, but can also affect other organs such as the skin, eyes, and central nervous system. Other symptoms may include fever, weight loss, fatigue, and night sweats. However, some individuals may remain asymptomatic.
DRDS primarily affects children and young adults, with a slight male predominance. The exact cause of the syndrome is unknown, but it is believed to be related to abnormal immune system responses. It is not considered an inherited condition.
Diagnosis of DRDS involves conducting thorough physical examinations, blood tests, imaging studies such as CT or MRI scans, and in some cases, a biopsy of the affected tissue. The characteristic feature of DRDS is the presence of histiocytes with specific cellular features observed under a microscope.
Currently, there is no specific cure for DRDS, and treatment usually focuses on managing the symptoms. In some cases, when the disease affects vital organs or causes significant discomfort, corticosteroids, chemotherapy drugs, or immunosuppressive medications may be employed. Surgical excision or radiotherapy may be considered for localized lesions. The prognosis is generally favorable, with most individuals experiencing spontaneous remission or a chronic but benign course. However, rare cases of aggressive or multi-organ involvement with a poorer prognosis have been reported.
