What is rdd disorder?

Rosai-Dorfman disease (RDD), first described in 1969, is a disease that occurs when the body produces too many histiocytes in the lymph nodes. These histiocytes most often accumulate in the nodes in the neck (cervical nodes), though other lymph nodes and other parts of the body may also be affected.

Is sinus histiocytosis cancerous?

Sinus histiocytosis is often associated with malignant tumor. It is considered as a sign of the host’s immune activation with favorable prognostic significance. Nevertheless, such lymphadenopathy mimics a metastatic lymph node, making proper staging difficult.

What is rdd in children?

Rosai-Dorfman disease is a rare disorder characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body (lymphadenopathy), most often those of the neck (cervical lymphadenopathy).

What is Sinus histiocytosis in a lymph node?

Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymph nodes with extra-nodal involvement in some cases. It is a self-limiting disease and has a good prognosis; however some patients need steroid therapy.

Where are histiocytes found?

A histiocyte is a normal immune cell that is found in many parts of the body especially in the bone marrow, the blood stream, the skin, the liver, the lungs, the lymph glands and the spleen. In histiocytosis, the histiocytes move into tissues where they are not normally found and cause damage to those tissues.

What is Castlemans disease?

Castleman disease is a rare disorder that involves an overgrowth of cells in your body’s lymph nodes. The most common form of the disorder affects a single lymph node (unicentric Castleman disease), usually in the chest or abdomen.

What is the significance of sinus histiocytosis?

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites. It usually shows a prolonged clinical course with occasional exacerbation and remission phases.

Is Rosai-Dorfman disease an autoimmune disease?

Rosai–Dorfman disease (RDD) is a rare non-neoplastic histioproliferative disorder characterised by painless lymphadenopathy, low fever, high erythrocyte sedimentation rate, leucocytosis and hypergammaglobulinaemia. Overactivity of nuclear factor κB (NF-κB) is linked with inflammatory, cancerous and autoimmune diseases.

What do histiocytes look like?

In smears, the typical histiocyte is easily identified by its size, eccentric round or bean-shaped nucleus, and lightly stained lacy cytoplasm (Fig. 19.43). Because histiocytes are phagocytic, their cytoplasm may contain leukocytes, nuclear particles (Fig.

Is Castlemans disease genetic?

Is Castleman disease inherited? Although the exact underlying cause of Castleman disease is unknown, it is thought to occur sporadically in people with no family history of the condition.