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A rare illness that affects the lymph nodes and other immune-cell structures of the body is referred to as Castleman disease.
Castleman disease is classified as a lymphoproliferative disorder meaning that it involves a proliferation or overgrowth of lymphatic cells. It is also known as giant lymph node hyperplasia and angiofollicular lymph node hyperplasia. Though Castleman disease is similar to cancers of the lymphatic system (lymphomas), which are also characterized by cell overgrowth, but this disease is not considered a cancer.
Castleman disease could occur in a widespread form or a localized one. Which type of Castleman disease a person has is the thing that determines treatment and outlook.
Here are the two basic types of Castleman disease and the different ways in which they affect people:
Unicentric Castleman disease:
Only a single lymph node is affected by this localized form of the disease. No symptoms are noticed at all in many people with this type of the disease. The diseased lymph node is located in the chest or abdomen in most cases. In case there are signs and symptoms, they may include:
Multicentric Castleman disease:
Multiple lymph nodes and lymphatic tissues are affected by this type, which can severely weaken the immune system. It sometimes accompanies HIV/AIDS. Systemic signs and symptoms develop in people with this type of the disease such as:
A person should talk to a doctor when noticing an enlarged lymph node on the side of the neck, or in the groin area, underarm or collarbone. When having other signs or symptoms such as unexplained weight loss, fatigue, fever or a feeling of fullness in the chest or abdomen, the person should also call the doctor. In many different types of illnesses, these signs and symptoms are common, so to determine the underlying cause, the person should see a doctor.
The exact cause of Castleman disease is still not known. Especially in multicentric Castleman disease, infection by a virus called the human herpes virus 8 (HHV8) is associated with Castleman disease, and it is suspected that it may play a causal role. This virus has been linked to the development of Kaposi's sarcoma as well, which is a cancerous tumor of the blood vessel walls and is common in people with multicentric Castleman disease as well. Both Kaposi sarcoma and Castleman disease can affect people who are HIV-positive.
Though it appears that HHV8 may help malfunctioning immune system cells reproduce rapidly, the precise role of it is not clear. A protein produced by the immune system cells – that are called interleukin-6 (IL-6) – may contribute to the overgrowth of lymphatic cells.
Via transplants, blood transfusions and sexual contact, the HHV virus may be passed, and it is likely transmitted through saliva.
Unicentric Castleman disease isn't associated with HHV8 in general.
Except for having HIV/AIDS, there doesn't seem to be any consistent risk factors for Castleman disease. Whereas most people with the multicentric form are in their 50s and 60s, the average age of people with unicentric Castlemand disease is around 30 or 40. Women and men, children and adults may all be affected by the disease.
Unicentric Castleman disease:
Although having Castleman disease may increase one's risk of lymphoma; once the affected lymph node is removed, people with unicentric Castleman disease usually do well.
Multicentric Castleman disease:
Conversely, multicentric Castleman disease is often life-threatening and it is much more serious. Failure of multiple organs, serious infection, or cancer such as Kaposi sarcoma or lymphoma, is usually the cause of death form multicentric disease. The outcome may worsen because of the presence of HIV.
The type of Castleman disease a person has is the thing that determines treatment. While muticentric Castleman disease requires more systemic therapies, treatment for unicentric Castleman disease is almost always with surgery.
UNICENTRIC CASTLEMAN DISEASE:
By surgically removing the diseased lymph node, unicentric Castleman disease can be cured. To remove the node, major surgery may be required if the lymph node is in the chest or abdomen, which is often the case with unicentic disease. A hospital stay is usually needed in this case. A simpler procedure may be performed that doesn't require hospitalization if the affected node is in a place that's easy to access, such as in the neck or underarm area.
Radiation therapy may be an effective way to destroy the affected tissue if surgical removal is not possible, such as in the case when the lymph node is difficult to get to.
MULTICENTRIC CASTLEMAN DISEASE:
Generally, it is more difficult to treat multicentric Castleman disease. It is difficult to identify the best treatment because the disease spontaneously goes into remission at times, has varied nonspecific signs and symptoms, and it is rare. Besides, there are no clinical trials that offer definitive evidence in favor of any therapy. Whether treatment should differ based on HIV status is also not clear.
Relieving signs and symptoms rather than curing the illness is the goal of most therapies, and this means that they are palliative. Although sometimes removing an enlarged spleen may help ease symptoms, but because of the number of lymph nodes involved, but in the case of multicentric disease, surgery usually is not an option.
To provide the most relief, doctors try as many therapies as they can in general. With varying degrees of success, therapies that are commonly used for multicentic disease include the following:
The activity of the virus HHV8 is inhibited by antiviral medications, such as ganciclovir. This therapy is effective for some people, but not for others, so its success has been mixed. Which antiviral is best and when is the best time to administer it, are still included in the questions that doctors have about antiviral therapy for Castleman disease.
Focusing on specific cells in the body that aren't working right is what these man-made antibodies are designed to do. By neutralizing or blocking the activity of IL-6 – the immune-cell protein suspected of playing a role in causing the disease – is how one such antibody that may be effective for multicentric Castleman disease works. Rituximab, a drug that targets a different type of cell associated with Castleman disease, is another monoclonal antibody that may help.
Because anti-cancer drugs (chemotherapy) target reproducing cells, such as those associated with castleman disease, they can be useful. Symptoms tend to return if chemotherapy is stopped, and in this case, to maintain symptom relief, further treatment is needed. Yet, for some people, chemotherapy has helped the disease go into remission. A combination of anti-cancer drugs is usually used. Reduced resistance to infection, loss of appetite, vomiting, nausea and hair loss may be included in the side effects of chemotherapy.
Doctors have tried immune modulators, which are drugs that help regulate the immune system, in the hopes that they may be effective. In a few people with Castleman disease, interferon alpha that has both immunoregulatory and antiviral effects, has been used successfully despite the fact that symptoms usually return once the patient stops taking the drug. The production of IL-6 may be decreased by thalidomide, which is a powerful immunomodulatory drug that has helped a few people.
Controlling inflammation is the basic function of corticosteroid therapy. Steroids such as prednisone are prescribed in combination with other therapies in the case of multicentric Castleman disease. Weak bones, weigh gain, increased blood pressure and reduced resistance to infection may all be the result of long-term use of steroids.
Dr. Tahsin Martini
Samir Moussa M.D.
Dr . Dirar Abboud
Dr. Faisal Dibsi
Dr. Talal Sabouni
Dr. Samer Al-Jneidy
Dr. Hani Najjar
Yaser Habrawi , F.R.C.S.Ed