Is Rosai-Dorfman disease a cancer?
Rosai-Dorfman disease is a benign disease which is characterized by over-production and accumulation of a specific type of white blood cell (histiocyte) in the lymph nodes of the body, most often those of the neck (cervical).
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.
Is Rosai-Dorfman curable?
In many cases, the symptoms of Rosai-Dorfman disease may disappear without treatment (spontaneous remission) within in months or a few years. Clinical observation without treatment is preferred for individuals with Rosai-Dorfman disease whenever possible. In many cases, no therapy will be necessary.
Is Rosai-Dorfman an autoimmune disease?
It is characterized by a non-clonal proliferation of distinctive cells of macrophage or histiocyte lineage, that primarily accumulate in lymph nodes [1]. We present 2 case reports in which patients were found to have an autoimmune disease as well as a rare, benign histiocytic disorder known as Rosai-Dorfman disease.
Is Rosai-Dorfman disease serious?
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. Histiocytes may also build up in the skin, upper respiratory tract, and sinuses. Rosai-Dorfman disease is seldom life-threatening.
Is histiocytosis fatal?
Langerhans cell histiocytosis can cause damage to tissues and organs all over the body if it’s not treated. One example is pulmonary histiocytosis. This condition damages the lungs. Damage to the body can be so severe that the condition becomes fatal.
How do you treat sinus histiocytosis?
The available information on treatment of sinus histiocytosis with massive lymphadenopathy (SHML) was reviewed. Treatment is not necessary in most instances, but some patients may require surgery, radiation therapy, and/or chemotherapy because of severe disease manifestations.
How is Rosai-Dorfman disease diagnosed?
Although the guidelines are focused on adult patients, there may be insight for pediatric physicians as well. The Guidelines are listed as “histiocytic neoplasms” and can be found on NCCN’s website. The diagnosis of Rosai-Dorfman (RD) is made following a biopsy of the affected tissue.
Is Rosai-Dorfman genetic?
Nevertheless, we propose that Rosai–Dorfman disease is a clonal histiocytic disorder driven by genetic alterations, at least in mutated cases, similar to Langerhans cell histiocytosis and other non-Langerhans cell histiocytosis histiocytoses.
Is Rosai-Dorfman disease genetic?
What is sinus histiocytosis?
Sinus histiocytosis with massive lymphadenopathy (SHML), is a benign, self-limiting disorder that commonly involves the lymph nodes.[1] First described by Rosai and Dorfman in 1969, many more cases have been added to the literature but the cytomorphology has been documented in relatively few cases.[2] It is …
What is malignant histiocytosis?
Malignant histiocytosis is an uncommon disease of dogs that is overrepresented in certain breeds, thereby underlining its heritability. It’s an aggressive, tragic disease that involves the abnormal accumulation of a type of white blood cell called the histiocyte.
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.
What does malignant histiocytosis look like?
Dogs with malignant histiocytosis are presented with nonspecific complaints of weight loss, lethargy, and inappetance. Other clinical findings include dyspnea, neurologic abnormalities, ocular signs, lymphadenopathy, hepatosplenomegaly, and anemia.
How do you treat Rosai-Dorfman?
Treatments and overall response rates (ORR) in patients with Rosai-Dorfman disease. Corticosteroids were used as the first-line therapy in 17 (27%) patients. Of these, responses were observed in in 56% of the cases, with a maximum response duration of 71 months and a relapse rate of 53%.
What are the symptoms of histiocytosis?
Symptoms
- Abdominal pain.
- Bone pain.
- Delayed puberty.
- Dizziness.
- Ear drainage that continues long-term.
- Eyes that appear to stick out more and more.
- Irritability.
- Failure to thrive.
What is Rosai Dorfman disease?
Rosai-Dorfman Disease (“Sinus histiocytosis with massive lymphadenopathy” or SHML) is a rare benign tumor characterized by an abundance of histiocytes (tissue resident macrophages, dendritic cells) in the involved tissue.