What is castleman disease? Causes, symptoms and treatment

Castleman’s disease also known as angiofollicular lymph node hyperplasia is a rare condition that involves an overgrowth of cells in the lymphatic system of our body. The growth can either be localized, affecting just one lymph node or a widespread that may affect two or more lymph nodes.

Both these types of Castleman’s diseases have varying symptoms and the associated treatments.

Unicentric Castleman’s Disease (UCD)

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The exact causes are not known clearly but some scientists believe that excessive production of the interleukin-6 by the cells in the lymph nodes gives rise to unicentric Castleman’s disease. Interleukin-6 is responsible for coordinating the response of the immune system to threats. But the excessive production creates an imbalance leading to UCD.

UCD may or may not have symptoms. In some people who show symptoms can exhibit it in a variety of ways depending on which lymph node has been affected. The enlarged lymph node is going to press against the nearby organs which may create discomfort in the chest or abdomen leaving a sensation of fullness even before eating a meal, excessive and unintentional weight loss, fever, night sweats, rashes on the skin, or weakness.

It is a common type of Castleman’s disease which usually affects the node in the abdomen or chest area. It can be cured by removing the affected node via a surgery. In cases where surgery is not possible or the lymph node cannot be removed completed, it is recommended that radiation therapy be used to remove or destroy the affected cells.

Multi-centric Castleman’s Disease (MCD)

Given that multi-centric Castleman’s disease affects more than one lymph node and is widespread, it becomes a complicated condition to handle. What causes MCD is not understood clearly but scientists suspect that the virus Human Herpes Virus 8 (HHV8) is linked with multi-centric Castleman’s disease. This virus seems to cause a malfunctioning in the cells of the immune system to rapidly produce cells.

MCD may make itself prominent through symptoms such as fever, enlarged lymph nodes, enlarged liver or spleen, rashes on the skin, nausea or vomiting, shortness of breath, etc.

The treatment for patients suffering from multi-centric Castleman’s disease depends on a variety of factors such as how widespread it is or its severity and if the patient is affected with HIV or HHV8 virus as well. The possible treatment usually involves the use of medication and some therapies to reduce the overgrowth. Surgical removal of the spleen mellows down the symptoms quite a bit. Of course, surgery to remove the lymph nodes is not an option due to the number of nodes involved in MCD.

A few treatment options are:

Immunotherapy

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Monoclonal antibodies are used to reduce the production of interleukin-6 and thereby reducing the excessive growth of the lymph nodes. This is used as an initial treatment.

Chemotherapy

If immunotherapy doesn’t show results or if there is organ failure, the doctor may recommend going for chemotherapy to destroy the overgrown cells and slow down further growth.

Corticosteroids

These steroids may be prescribed to contain inflammation.

Thalidomide

It is an immune system modular that has the capacity to block the working of interleukin-6. This has shown some results by reducing the severity of the disease.

Anti-viral drugs

Those patients with HIV or HHV8 disease have known to benefit from these drugs.

Complications

Once affected by Castleman’s diseases life can become a little challenging for the patient as this disease can lead to various other diseases and complications. Castleman’s disease is known to increase the chances of getting affected by lymphoma, a type of cancer. Complications from MCD may lead to multiple organ failures, making dealing with Castleman’s disease more and more complex.