Benign giant lymph node hyperplasia: Description, Causes and Risk Factors:
Benign giant lymph node hyperplasia is a disease of lymph nodes and related tissues. Benign giant lymph node hyperplasia is not officially a cancer, but the multicentric form of this disease acts very much like lymphoma (cancer of lymph nodes). In fact, many people with this disease eventually develop lymphomas.
Localized benign giant lymph node hyperplasia: Localized or unicentric benign giant lymph node hyperplasia only affects a single set of lymph nodes. It is not widespread. The lymph nodes in the chest and abdomen are affected most often. These abnormally large lymph nodes may press on other organs and tissues inside the chest or abdomen. If they are in the abdomen, the person might feel pain or pressure in that area. Enlarged lymph nodes in the chest can press on the windpipe (trachea) or smaller breathing tubes (bronchi) causing breathing problems. Sometimes the enlarged lymph nodes are in places such as the neck, groin, or underarms and can be felt easily. People with localized benign giant lymph node hyperplasia disease are usually cured when the lymph node is removed with surgery.
- Multicentric benign giant lymph node hyperplasia: Multicentric benign giant lymph node hyperplasia affects more than a single group of lymph nodes. It can also affect other organs containing lymphoid tissue. This form sometimes occurs in people infected with human immunodeficiency virus (HIV), the virus that causes AIDS. Multicentric benign giant lymph node hyperplasia is more serious than the localized type, particularly in people with HIV infection. People with multicentric benign giant lymph node hyperplasia often have serious infections, fevers, weight loss, fatigue, night sweats, and nerve damage that can cause weakness and numbness. Blood tests often show too few red blood cells (anemia) and high levels of antibodies in the blood (called hypergammaglobulinemia). They also show signs of malnutrition. Benign giant lymph node hyperplasia can weaken the immune system severely, making it hard to fight infection. Infections in people with multicentric benign giant lymph node hyperplasia can be very serious and may even lead to death. Benign giant lymph node hyperplasia also increases the risk of developing lymphoma, a cancer of lymphoid tissue. This can be fatal.
There are no known causes of this disease, but many doctors suspect a virus is involved. Doctors have found a virus called human herpes virus type 8 (HHV-8) in many people with multicentric benign giant lymph node hyperplasia. This virus is also known as Kaposi sarcoma--related herpes virus (KSHV) because it has also been found in people with Kaposi sarcoma. In fact, some people with benign giant lymph node hyperplasia also have Kaposi sarcoma.
Doctors suspect that problems with the way a patient's immune system is working may contribute to the development of benign giant lymph node hyperplasia. Many people with benign giant lymph node hyperplasia have abnormally high blood levels of certain substances produced by immune system cells.
Some scientists believe that benign giant lymph node hyperplasia occurs when there is too much of a protein called interleukin-6 (IL-6). IL-6 is a protein the body makes to help regulate immune function. Too much IL-6 seems to cause lymphocytes to reproduce excessively. High levels of IL-6 are seen in benign giant lymph node hyperplasia, and tend to be associated most often with multicentric disease. There is a link between HHV-8 and IL-6.
The most common include:
- Weakness and fatigue (tiredness).
- Night sweats.
- Weight loss.
- Loss of appetite.
- Nausea and vomiting.
- Nerve damage that leads to numbness and weakness (neuropathy).
- Leg swelling (edema).
- Skin rashes.
Diagnosis may include:
Computed Tomography: The computed tomography or CT scan is an x-ray procedure that produces detailed cross-sectional images of your body. Instead of taking one picture like a conventional x-ray, aCT scanner takes many pictures as it rotates around you. A computer then combines thesepictures into an image of a slice of your body. The machine takes pictures of multipleslices of the part of your body that is being studied. This test can help tell if benign giant lymph node hyperplasia is in only one lymph node or many.
Before the first set of pictures is taken you may be asked to drink 1 or 2 pints of acontrast agent. This helps outline the intestine so that certain areas are not mistaken fortumors. You may also receive an intravenous (IV) line through which a different kind ofcontrast dye is injected. This helps better outline structures in your body.
The injection can cause some flushing (redness and warm feeling that may last hours todays). A few people are allergic to the dye and get hives. Rarely, more serious reactionslike trouble breathing and low blood pressure can occur. You can be given medicine toprevent and treat allergic reactions. Be sure to tell your doctor if you have ever had areaction to any contrast material used for x-rays.
CT scans take longer than regular x-rays because you need to lie still on a table whilethey are being done. Also, you might feel a bit confined by the equipment you lie withinwhile the pictures are being taken.
CT scans can also be used to guide a biopsy needle precisely into a lymph node that isenlarged from things like infection, lymphoma, metastatic cancer, or benign giant lymph node hyperplasia. For thisprocedure, called a CT-guided needle biopsy, the patient remains on the CT scanningtable 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 lymphnode. A fine needle biopsy sample (tiny fragments of tissue) or a core needle biopsysample (a thin cylinder of tissue about -inch long and less than 1/8-inch in diameter) isremoved and examined under a microscope. A needle biopsy cannot accurately diagnosebenign giant lymph node hyperplasia by itself, it can sometimes help diagnose or exclude other diseases that can causelarge lymph nodes.
Magnetic Resonance Imaging: Magnetic resonance imaging (MRI) scans use radio waves and strong magnets instead ofx-rays. The energy from the radio waves is absorbed and then released in a patternformed by the type of body tissue and by certain diseases. A computer translates thepattern of radio waves given off by the tissues into a very detailed image of parts of thebody. Not only does this produce cross-sectional slices of the body like a CT scanner, itcan also produce slices that are parallel with the length of your body. A contrast materialmight 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 littlemore uncomfortable than CT scans. First, they take longer -- often up to an hour. Also,you have to be placed inside tube-like equipment, which is confining and can upsetpeople with a fear of enclosed spaces. The machine also makes a thumping noise that youmay find disturbing. Some places provide headphones with music to block this out.
Chest X-ray: This may be done to find out whether there are enlarged lymph nodes in your chest --usually in the center part called the mediastinum.
Gallium Scan: For this test, the radiologist injects a radioactive chemical called gallium into a vein. Thechemical is attracted to areas of the body affected by certain diseases such as lymphomaor benign giant lymph node hyperplasia. A special camera can then locate the gallium. A gallium scan can findunsuspected sites of benign giant lymph node hyperplasia disease, but it is not always reliable since the radioactive galliummay not be taken up by all of the lymph nodes affected by benign giant lymph node hyperplasia.
Positron Emission Tomography: Positron emission tomography (PET) scans are helpful in finding small collections ofcancer cells that may not be visible on CT scan. PET is not often used to diagnose benign giant lymph node hyperplasia,but sometimes it can be helpful. In the PET scan, radioactive glucose (sugar) is injectedinto the patient's vein. Because cancer cells use sugar much faster than normal cells, theradioactive material builds up in cancer cells. A scanner is used to spot the radioactivedeposits. CD cells don't take up glucose as much as cancer cells, but they do seem to takeit up more than normal cells. Often the PET scan is combined with a CT scan. This helpsdecide if abnormalities seen on the CT scan are CD, cancer, or something else.
Lymph Node Biopsy: Benign giant lymph node hyperplasia can only really be diagnosed by removing the enlarged lymph nodeand examining it under the microscope. This procedure is called a biopsy. If the lymphnode is near the skin surface, the surgeon can remove the node using local anesthesia(numbing medicine). The surgeon makes a small incision (cut) over the enlarged lymphnode, removes the node, and then stitches the incision closed. If the procedure removesthe entire lymph node, it is called an excisional biopsy. If only part of the node isremoved, it is called an incisional biopsy.
Sometimes lymph nodes are biopsied by putting a needle into the node. A very thinneedle can remove tiny fragments of tissue; this is called a fine needle aspiration biopsy.A core needle biopsy uses a slightly larger needle to remove a cylinder-shaped core oftissue. Doctors have reported that diagnosis of benign giant lymph node hyperplasia by needle biopsy is sometimespossible, but biopsy methods that remove larger samples of tissue are usuallyrecommended because they are considered more accurate.
If the lymph node is in the chest or the abdomen, then the surgeon may need to make alarge incision to get into either of these places. This is more like major surgery but it maybe necessary to know what is causing the lymph node to enlarge. Sometimes, lymphnodes in the chest can be removed by mediastinoscopy. In this procedure, the surgeonuses a hollow tube that has a camera on the end (called a mediastinoscope), which isinserted through a small incision just above the breastbone (sternum). Special instrumentsinserted through the scope can be used to biopsy lymph nodes.
All biopsy specimens are examined under a microscope by a pathologist (a doctor who isspecially trained to diagnose disease). The pathologist looks at the size, shape, andarrangement of the cells in the lymph node. Since the disease is so rare, the pathologistmay ask another pathologist with additional training in the diagnosis of blood and lymphnode diseases (called a hematopathologist) to look at the biopsy. Sometimes it is hard totell if the lymph node is affected by benign giant lymph node hyperplasia or by lymphoma. In these cases, other tests maybe done on the lymph node tissue.
Immunohistochemistry: In this test, a part of the biopsy sample is treated with special man-made antibodies. Thecells are treated so that certain types of cells change color. The color change can be seenunder a microscope. It may help tell whether there is benign giant lymph node hyperplasia or lymphoma in the lymphnode.
Flow Cytometry: Cells from the lymph node are treated with special manmade antibodies and passed infront of a laser beam. Each antibody sticks only to certain types of cells. If the samplecontains those cells, the laser light causes them to give off light of a different color. Theintensity of each color is measured exactly and analyzed by a computer. This test canhelp determine whether lymph node swelling is caused by lymphoma, some other cancer,or a non-cancerous disease like benign giant lymph node hyperplasia
Surgery: Surgery is often used to obtain a tissue sample to diagnose benign giant lymph node hyperplasia. A lymph node biopsy is usually a minor procedure. Surgery also works well to treat localized benign giant lymph node hyperplasia. The type of surgery depends on where the disease is located. When the enlarged lymph node is in a place that is hard to get to, like deep in the chest or abdomen, it means surgery in those areas. These are common procedures, but they are serious. The patient often has some pain and may need to stay in the hospital for a few days after the operation. If the involved lymph nodes are in a place that is easy to get to, such as in the armpit, then surgery is simpler, there is less pain after the operation, and hospitalization may not be needed.
Radiation Therapy: Radiation therapy uses high-energy radiation to kill cells. Radiation focused from a source outside the body is called external beam radiation. Radiation therapy has sometimes been used instead of surgery to treat localized disease.
Side effects of radiation therapy may vary but often include mild skin problems and fatigue. Radiation of the abdomen may cause nausea, diarrhea, and loss of appetite. These side effects tend to improve a short while after the radiation is stopped. When radiation therapy is given to the chest, there is a risk of permanent lung damage leading to breathing problems and shortness of breath. Radiation may also make the side effects of chemotherapy worse if they both are given at the same time.
Corticosteroids: Corticosteroids are a group of drugs related to hormones produced by the adrenal glands. These drugs are useful in treating people with certain immune system diseases and cancers that develop from immune system cells, such as lymphomas. Some patients with multicentric benign giant lymph node hyperplasia benefit from treatment with these drugs.
Corticosteroids are often taken as pills, but they can also be given as an injection into a vein. Prednisone, a corticosteroid pill, is often used to treat lymphoma and CD.
Side effects of corticosteroids can include increased blood sugar that may lead to diabetes, depression, reduced resistance to infections, weakened bones, fatigue, muscle weakness, weight gain, fluid retention, and high blood pressure. Most of these side effects improve after the drug is stopped.
Chemotherapy: Chemotherapy (chemo) is the use of anti-cancer drugs that are injected into a vein or a muscle or are taken by mouth. These drugs enter the bloodstream and reach all areas of the body, making this treatment very useful for multicentric benign giant lymph node hyperplasia. Chemo may be used alone, in combination with corticosteroids, or in combination with radiation therapy (this is called chemoradiation).
Many drugs have been used to treat patients with benign giant lymph node hyperplasia. Those used most often include carmustine, cladribine, chlorambucil, cyclophosphamide, doxorubicin, etoposide, melphalan, vinblastine, and vincristine. Often several drugs are combined. Chemotherapy combinations like those used for lymphoma have been used. Depending on the drugs, the treatments are given on different schedules but are usually repeated several times in cycles 3 or 4 weeks apart.
Many chemotherapy treatments are given on an outpatient basis (in the doctor's office or clinic), but some require hospital admission. Sometimes a patient takes one drug combination for several cycles and then later is switched to a different one. Because multicentric benign giant lymph node hyperplasia is so rare, there is not a lot of information on which chemo treatment is best or even how well it works.
Chemotherapy drugs can damage normal cells, leading to some side effects. These depend on the type and dose of drugs given and the length of time they are taken. Drugs used in chemotherapy attack cells that are rapidly dividing. This means they will also attack some normal tissues such as the bone marrow, the lining of the mouth and intestines, and the hair follicles, which also grow rapidly to replace cells that wear out. As a result, a patient may have: hair loss, mouth sores, loss of appetite, nausea and vomiting, lowered resistance to infection (due to low white blood cell counts), easy bruising and bleeding (due to low platelet counts), fatigue and weakness (due to anemia - low red blood cells). Your doctor will try to avoid or lessen these side effects as much as possible. For example, drugs can be given before or along with chemotherapy to prevent or reduce nausea and vomiting. Most side effects are temporary and go away after treatment is finished.
A low white blood cell count is an important risk factor for serious infections, so some patients find it helpful to keep track of their counts. If you are interested in this information, ask your doctor or nurse about your blood cell counts and what these numbers mean. You may want to keep a diary of your treatment and blood counts to help you follow the effects of your treatment.
Organs that could be damaged by chemotherapy drugs include the kidneys, liver, testes, ovaries, brain, heart, and lungs. Many of the drugs used to treat benign giant lymph node hyperplasia can cause nerve damage, leading to problems such as numbness and tingling in the hands and feet. If serious side effects occur, the chemotherapy may have to be reduced or stopped, at least temporarily. Your doctor will carefully monitor and adjust drug doses because some side effects can be permanent.
Monoclonal antibodies: Monoclonal antibodies are special immune proteins made in the lab. They are directed toward specific molecules on the surface of cells. Rituximab (Rituxan®) is a monoclonal antibody that is widely used for lymphoma. It can also be helpful in treating benign giant lymph node hyperplasia. Rituximab specifically recognizes and attaches to a protein called CD20 that is found on the surface of some lymphocytes. This attachment tells the cell to die. Patients get rituximab through infusion into a vein (IV) at the oncologist's office or clinic. Side effects are most common during the infusion, and include chills, fever, nausea, rashes, fatigue, and headaches. Unlike regular chemotherapy, rituximab does not cause low blood counts or hair loss.
Thalidomide: The drug thalidomide is a type of drug called an immunomodulating agent. It is used to treat multiple myeloma, and has helped some patients with benign giant lymph node hyperplasia. Side effects of thalidomide include drowsiness, fatigue, severe constipation, and neuropathy (nerve damage causing pain). There is also an increased risk of serious blood clots that start in the leg and can travel to the lungs. Because thalidomide causes severe birth defects if taken during pregnancy, this drug can only be obtained through a special program run by the drug company that makes it.
Interferon: Interferon is a hormone-like protein naturally produced by white blood cells to help the immune system fight infections. Some patients with benign giant lymph node hyperplasia disease have improved with interferon treatment. Side effects of this treatment include moderate to severe fatigue, fever, chills, headaches, muscle and joint aches, and mood changes.
Antiviral drugs: Because benign giant lymph node hyperplasia is associated with the virus HHV-8, doctors have had some success in treating a few patients with multicentric benign giant lymph node hyperplasia with drugs that kill this virus.
Clinical trials: You may have had to make a lot of decisions since you have been told you have benign giant lymph node hyperplasia. One of the most important decisions you will make is choosing which treatment is best for you. You may have heard about clinical trials being done for benign giant lymph node hyperplasia or maybe someone on your health care team has mentioned a clinical trial to you.
Clinical trials are carefully controlled research studies that are done with patients who volunteer for them. They are done to get a closer look at promising new treatments or procedures. If you would like to take part in a clinical trial, you should start by asking your doctor if your clinic or hospital conducts clinical trials. You can also call our clinical trials matching service for a list of clinical trials that meet your medical needs.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.