Castleman Disease

### What is the lymphatic system?

The lymphatic system is the network of tissues and organs that help get rid of the body toxins, waste and unwanted materials. The main function of this system is to transport lymph, which is a fluid that contains infection-fighting white blood cells throughout the entire body.

### What is Castleman disease?

Castleman disease (CD) is a rare disease of lymph nodes and related tissues. It is not a cancer, it is called a *lymphoproliferative disorder.* Basically, there is an abnormal overgrowth of cells of the lymph system that is similar to *lymphomas*, which is cancer of lymph nodes. The importance is that CD is not officially a cancer, but one form of the disease acts very much like *lymphoma.* Many people with CD eventually develop *lymphomas*, and for this reason, Castleman disease is included in the American Cancer Society’s cancer information. CD can affect children as well as adults, typically younger people are more likely to have the localized form of CD.

### How does Castleman disease affect the lymphatic system?

Castleman disease is essentially overgrowth in the lymph nodes. When the lymph nodes begin to increase in size ‘hyperplasia’. Hyperplasia occurs in the lymph nodes and lymphatic tissues area. Since the lymphatic system is made up of tissues and organs that help get rid of toxins, waste and unwanted material, when these enlarge they become swollen. This is why the symptoms that people experience are painful.  Since Castleman disease is overgrowth in the lymph nodes, the lymphatic system is being clogged presenting the symptoms.

### Signs and Symptoms

Castleman disease can cause many different symptoms and in some peoples, it might not cause any symptoms at all. If symptoms do occur they are often seen for other diseases like; infections, autoimmune diseases, or even some types of cancer. This is why doctors might not suspect CD at first. The common symptoms included enlarged lymph node in the chest which can press on the windpipe, resulting in trouble breathing, wheezing, coughing or a feeling of fullness in the chest. Also, a symptom could be an enlarged node in the abdomen which can cause trouble eating, pain or just a feeling of fullness.

### Diagnosis

 People with Castleman disease will often go to the doctor because of the symptoms they are experiencing. Sometimes CD is found in people without symptoms when it’s seen on an imaging test being for other reasons. Since CD is rare and the symptoms are often like those caused by other diseases doctors often suspect it is something else at first. The actual diagnosis of CD is made when doctors remove an affected lymph node and look at it under a microscope. Essentially, the patient would need a *biopsy*. Also, the patient will likely undergo blood tests, even though they will not be able to tell in the patient has CD. Imaging tests are done to look for enlarged lymph nodes or organs that might be causing symptoms, to look for enlarged lymph nodes in other parts of the body and to help determine if treatment is working affectively.

### Treatment

Castleman disease is often treated with chemotherapy or radiation therapy as it is believed to be part of cancer. Once you are diagnosed with Castleman disease (CD), your health representative will discuss different types of treatment used for CD. The following are several forms of treatment; surgery, radiation therapy, corticosteroid drugs, chemotherapy, immunotherapy, and anti-viral drugs. The treatment options will be based on whether the CD is localized or metacentric, and other factors are kept in mind. Since CD is so rare many doctors do not have much experiences of treating it.

#####