An enlarged lymph node, usually inside the chest or abdomen, is the only abnormality for most people with the localized form of Castleman disease. They may have trouble breathing, a cough, or a feeling of fullness in the chest. Those with Castleman disease in their abdomen may have trouble eating, pain, or just a feeling of fullness. Some patients may have low grade fever, weight loss, or night sweats. In general, most people with localized Castleman disease feel well otherwise.
People with multicentric Castleman disease have more than one enlarged lymph node. The involved nodes may be in the chest or abdomen, but multicentric Castleman disease often affects lymph nodes in the groin, the underarm area, and on the sides of the neck. Multicentric Castleman disease can also affect lymphoid tissue of internal organs, causing the liver, spleen, or other organs to enlarge.
In addition, people with either type of Castleman disease may have other symptoms. The most common include:
- fever
- weakness
- night sweats
- weight loss
- loss of appetite
- nausea and vomiting
- nerve damage that leads to numbness and weakness
These symptoms occur much more often in multicentric than in localized Castleman disease.
Amyloidosis, a disease where abnormal proteins are deposited in tissues, can occur in Castleman disease. This can lead to kidney damage, heart damage, nerve damage, and intestinal problems, mainly diarrhea. Amyloidosis can go away if the Castleman disease is successfully treated.
Imaging Studies
Computed tomography (CT): The CT scan is an x-ray procedure that produces detailed cross-sectional images of your body. Instead of taking one picture, as does a conventional x-ray, a CT scanner takes many pictures as it rotates around you. A computer then combines these pictures into an image of a slice of your body. The machine takes pictures of multiple slices of the part of your body that is being studied. This test can help tell if your Castleman disease is in only one lymph node or many. Often after the first set of pictures is taken you will receive an intravenous (IV) injection of a contrast agent, or dye, that helps better outline structures in your body. A second set of pictures is then taken.
CT scans can also be used to guide a biopsy needle precisely into enlarged lymph nodes that might be affected by an infection, lymphoma, metastatic cancer, or Castleman disease. For this procedure, called a CT-guided needle biopsy, the patient remains on the CT scanning table while a radiologist advances a biopsy needle toward the location of the lymph node. CT scans are repeated until the doctors are confident that the needle is within the lymph node. A fine needle biopsy sample (tiny fragments of tissue) or a core needle biopsy sample (a thin cylinder of tissue about ?-inch long and less than 1/8-inch in diameter) is removed and examined under a microscope. Although a needle biopsy cannot accurately diagnose Castleman disease by itself, it is sometimes useful in diagnosing or excluding other diseases that can cause large lymph nodes.
CT scans take longer than regular x-rays because you need to lie still on a table for 15 to 30 minutes while they are being done. But just like other computerized devices, they are getting faster and your stay might be pleasantly short. Also, you might feel a bit confined by the equipment you lie within while the pictures are being taken.
You will need an IV line through which the contrast dye is injected. The injection may cause some flushing. Some people are allergic and get hives, or rarely more serious reactions like trouble breathing and low blood pressure can occur. Be sure to tell the doctor if you have ever had a reaction to any contrast material used for x-rays. You may also be asked to drink 1 to 2 pints of a contrast solution. This helps outline the intestine.
Magnetic resonance imaging (MRI): MRI scans use radio waves and strong magnets instead of x-rays. The energy from the radio waves is absorbed and then released in a pattern formed by the type of body tissue and by certain diseases. A computer translates the pattern of radio waves given off by the tissues into a very detailed image of parts of the body. Not only does this produce cross-sectional slices of the body like a CT scanner, it can also produce slices that are parallel with the length of your body. A contrast material might be injected just as with CT scans but is used less often.
MRI scans are very helpful in looking at the brain and spinal cord. MRI scans are a little more uncomfortable than CT scans. First, they take longer ??鈥?often up to an hour. Also, you have to be placed inside tube-like equipment, which is confining and can upset people with a fear of enclosed spaces. The machine also makes a thumping noise that you may find disturbing. Some places provide headphones with music to block this out.
Chest x-ray: This test may be done to find out whether there are enlarged lymph nodes in your chest ??鈥?usually in the center part called the mediastinum.
Gallium scan: For this test, the radiologist injects a radioactive chemical called gallium into a vein. The chemical is attracted to areas of the body affected by certain diseases such as lymphoma or Castleman disease. A special camera can then view the location of the gallium. A gallium scan can detect unsuspected sites of Castleman disease, but it is not always reliable since the radioactive gallium may not be taken up by lymph nodes affected by Castleman disease.
Lymph Node Biopsy
Castleman disease is diagnosed by removing the enlarged lymph node and examining it under the microscope. This procedure is called a biopsy. If the lymph node is near the skin surface, the surgeon can remove the node under local anesthesia (using numbing medication). The surgeon makes a small incision over the enlarged lymph node, removes the node, and then stitches the incision closed. If the procedure removes the entire lymph node, it is called an excisional biopsy. If only part of the node is removed, it is called an incisional biopsy.
Sometimes lymph nodes are biopsied by putting a needle into the node. A very thin needle can remove tiny fragments of tissue, and a slightly larger needle can remove a cylinder-shaped core or tissue. Although needle biopsies are useful for diagnosing some types of cancers, it is not possible to accurately diagnose Castleman disease by needle biopsies alone.
If the lymph node is in the chest or the abdomen, then the surgeon may need to make a large incision to get into either of these places. This is more like major surgery but may be necessary in order to know what is causing the lymph node to enlarge. Sometimes, lymph nodes in the chest can be removed by mediastinoscopy. In this procedure, the surgeon can biopsy the lymph nodes with special instruments through a hollow tube called a mediastinoscope, which is inserted through a small incision just above the breastbone (sternum).
All biopsy specimens are examined under a microscope by a pathologist (a doctor who is specially trained to diagnose disease). The pathologist looks at the size, shape, and arrangement of the cells in the lymph node. Since the disease is so rare, the pathologist may ask another pathologist with additional training in the diagnosis of blood and lymph node diseases (called a hematopathologist) to look at the biopsy. Sometimes it is hard to tell whether the lymph node is affected by Castleman disease or by lymphoma. In these cases, other tests may be done on the lymph node tissue.
Immunohistochemistry: In this test, a part of the biopsy sample is treated with special manmade antibodies. The cells are treated so that certain types of cells change color. The color change can be seen under a microscope. It may be helpful in telling whether there is Castleman disease or lymphoma in the lymph node.
Flow cytometry: The cells being examined by this test are treated with special manmade antibodies and passed in front of a laser beam. Each antibody sticks only to certain types of cells. If the sample contains those cells, the laser light causes them to give off light of a different color. The intensity of each color is measured exactly and analyzed by a computer. This test can help determine whether lymph node swelling is caused by lymphoma, some other cancer, or a noncancerous disease like Castleman disease.