Rosai–Dorfman disease

Rosai-Dorfman Disease in Elderly Female - Aziz Et Al Case Rep Pathol 2012

Rosai–Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare disorder characterized by an overproduction and accumulation of a specific type of white blood cell called histiocytes. These cells can accumulate in the lymph nodes and other tissues throughout the body.

History

Rosai–Dorfman disease was first described in 1969 by pathologists Juan Rosai and Ronald Dorfman. The disease is named after these two physicians who identified and characterized the condition.

Pathophysiology

The exact cause of Rosai–Dorfman disease is unknown. It is believed to be a disorder of the immune system, but the precise mechanisms are not well understood. The disease is characterized by the accumulation of histiocytes, which are a type of immune cell that normally helps to fight infection. In RDD, these cells accumulate abnormally in the lymph nodes and other tissues, leading to the characteristic symptoms of the disease.

Clinical Features

The most common symptom of Rosai–Dorfman disease is painless, massive lymphadenopathy, particularly in the neck. Other symptoms can include fever, night sweats, weight loss, and fatigue. In some cases, the disease can affect other organs, including the skin, central nervous system, and bones.

Diagnosis

Diagnosis of Rosai–Dorfman disease typically involves a combination of clinical evaluation, imaging studies, and biopsy of affected tissues. Histological examination of the biopsy sample reveals characteristic features, including the presence of large histiocytes with abundant cytoplasm and the phenomenon of emperipolesis, where intact lymphocytes are found within the cytoplasm of histiocytes.

Treatment

There is no standard treatment for Rosai–Dorfman disease, and the approach to management can vary depending on the severity and extent of the disease. In many cases, the disease is self-limiting and may not require treatment. When treatment is necessary, options can include corticosteroids, chemotherapy, radiation therapy, and surgery to remove affected lymph nodes or other tissues.

Prognosis

The prognosis for individuals with Rosai–Dorfman disease is generally good, especially in cases where the disease is limited to the lymph nodes. However, the prognosis can be more variable in cases where the disease affects other organs.

Related Pages

• Lymphadenopathy
• Histiocyte
• Immune system
• Juan Rosai
• Ronald Dorfman