Castleman's disease is a lymph node disorder associated with a number of viral infections. It is an extremely rare disorder, but patients afflicted with it have exceptionally high rates of many forms of cancer. So, what is Castleman disease pathology?
It is often associated with HIV and other sexually transmitted diseases, but Castleman's disease is not considered a sexually transmitted disease. Research into Castleman's disease is focused on finding out how this disease develops and how it promotes the development of cancer.
The symptoms associated with Castleman's disease are quite severe. Night fevers, intense sweating, dehydration, and inflammation are some of the most common. More severe symptoms such as death or organ failure are somewhat less common, but are still seen quite often. The severity of Castleman's disease makes this one of the most devastating diseases of the immune system.
In addition to the symptoms of the disease itself, there is a strong connection between Castleman's disease and viral infections. Many people with the disorder contract other diseases such as Karsi's sarcoma, a form of blood cancer often associated with HIV and lymphoma. This produces even greater problems for those with Castleman's disease.
Castleman's disease is generally regarded as a hereditary disease, although the exact cause of it is not known. Regardless of its origins, Castleman's disease is associated with a number of viruses. The viruses most often associated with Castleman's disease are within the herpes family. Particularly, the HHV8 virus is strongly linked to the disease. HHV8 is known for its connection to HIV and Karsi's sarcoma, but it shares a particularly special relationship with a specific form of Castleman's disease known as multicentric Castleman's disease.
While unicentric Castleman's disease, a form of the disease in which lymph nodes show changes at only one site, is considered a relatively benign form. Multicentric Castleman's disease, the form of the disease associated with changes in multiple sites, is much more severe, with death being a common result of the disease. Multicentric Castleman's disease is strongly associated with HHV8. HHV8 introduces new challenges to doctors treating Castleman's disease. Although antiviral medications can help fight it to a certain extent, the HHV8 virus is simply too strong most of the time.
The treatment options available to fight Castleman's disease vary greatly depending on whether we are referring to unicentric or multicentric forms of the disease. Unicentric Castleman's disease is usually treated through simple surgical removal, but multicentric forms of the disease often demand a more complicated approach. The presence of HHV8 may also determine what method is best suited for treatment.
If the patient is infected with HHV8, antiviral treatments are used to fight the disease. Drugs specifically designed to target herpes viruses such as ganciclovir are often used, but in many cases drugs specifically designed to attack the body's natural cells are used. If HHV8 isn't present but the patient still has the multicentric form of the disease, the doctor will typically use a series of corticosteroids to combat the symptoms. Some antiviral medications are used to combat the disease, but their effectiveness is disputed.
The exact cause of Castleman's disease is not known for certain. Given the strong association between Castleman's disease and viral infections, most doctors believe there is likely some sort of virus behind it. However, others believe the disease is likely hereditary in nature or influenced by mutations.
The fact that Castleman's disease patients frequently acquire forms of cancer has lead many researchers to believe it likely has a relationship to tumor suppressing genes. A malfunction in genes responsible for preventing the development of tumors in the lymphatic system could result in the abnormalities present in Castleman's disease patients.
Research into the disease is ongoing with every possible angle explored. The efforts to better understand this disorder will require much more energy than currently seen. Rare diseases don't often receive the proper treatment they deserve, but key insights into every disease helps medical knowledge as a whole.