Description
Castleman disease is a rare disease that affects the lymph nodes and immune cell structure. Also known as giant lymph node hyperplasia and lymph node hyperplasia angiofollicular. Castleman disease is classified as a lymphoproliferative disorder. These conditions mean involves the overgrowth (proliferation) of lymphatic cells. It is making Castleman disease similar to cancer lymphatic system (lymphoma), which is also characterized by an overgrowth of cells. Castleman disease is not considered cancerous, but was associated with a higher risk of this type of cancer called lymphoma. Castleman disease may occur locally or wider shape. Treatment depends on the type of Castleman disease.
Cause
It is unclear what caused Castleman disease. Infection by a virus called human herpesvirus 8 (HHV-8) associated with Castleman's disease, and doctors suspect that it may play a role, especially multicentric Castleman disease. This virus has also been associated with the development of Kaposi's sarcoma, cancerous tumors of the blood vessel wall, which is also common in people with multicentric Castleman disease. People who are HIV positive are more likely to have Castleman disease and Kaposi's sarcoma.
The researchers could not explain the role of HHV-8, but it seems that the virus can cause malfunctioning of the cells of the immune system to reproduce quickly. Immune cells produce a protein called interleukin 6 (IL-6) that contributes to the overgrowth of lymphatic cells and leads to a lot of signs and symptoms of Castleman disease. Generally, people with unicentric Castleman's disease is not infected with HHV-8.
Symptom
Unicentric Castleman disease
Many people with unicentric Castleman's disease, there are no symptoms at all. Most often, sore lymph nodes located in the chest or abdomen. When there are signs and symptoms, may include:
1. A feeling of fullness or pressure in the chest or abdomen that can cause difficulty breathing or difficulty eating
2. An enlarged lumps under the skin of the neck, groin, or armpits
3. Weight loss
4. Cough
5. Anemia
Multicentric Castleman disease
People with multicentric Castleman disease usually develops signs and symptoms may include:
1. Fever
2. Night sweats
3. Loss of appetite
4. Nausea and vomiting
5. Weight loss
6. Weakness
7. Fatigue
8. Enlarged peripheral lymph nodes, usually around the neck, collarbone, armpits, and groin
9. Enlarged liver or spleen
10. Nerve damage in hands and feet leading to numbness or weakness (peripheral neuropathy)
Treatment
Treatment depends on the type of Castleman's disease, which is owned. Treatment for unicentric Castleman disease is almost always with surgery, whereas multicentric Castleman's disease requires a more systemic therapies.
1. Unicentric Castleman disease
Unicentric Castleman disease can be cured by removal of diseased lymph nodes. If the lymph nodes in the chest or abdomen, which often occurs with unicentric Castleman's disease, major surgery may be required to take a node. If the nodes are affected in an easily accessible place, like in the armpit or neck, a simple procedure can be performed that do not require hospitalization. If surgical removal is not possible, as if lymph nodes are difficult to obtain, drugs, such as the monoclonal antibody rituximab (Rituxan) or corticosteroids, may be used to shrink lymph nodes. Radiation therapy also can be an effective way to destroy the affected tissue.
2. Multicentric Castleman disease
Treating multicentric Castleman disease is generally more difficult. Because the disease is rare, has a variety of nonspecific signs and symptoms. Doctors have found it difficult to identify the best treatment. Surgery is usually not an option for multicentric Castleman's disease as the number of lymph nodes involved, although sometimes remove an enlarged spleen may help reduce symptoms.
Drugs commonly used for multicentric Castleman's disease, with varying degrees of success. Doctors usually tried as a treatment as much as they can to provide the most appropriate assistance. Therapy can include:
1. Monoclonal Antibodies
2. Corticosteroids
3. Chemotherapy
4. Antiviral drugs
5. Immune modulators
Sources: MayoClinic
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