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updated July 11, 2009

Hodgkin's lymphoma (Hodgkin's disease)

Filed under: Cancer & Chemo
Hodgkin's lymphoma — formerly known as Hodgkin's disease — is a cancer of the lymphatic system, which is part of your immune system.

In Hodgkin's lymphoma, cells in the lymphatic system grow abnormally and may spread beyond the lymphatic system. As Hodgkin's lymphoma progresses, it compromises your body's ability to fight infection.

Hodgkin's lymphoma is one of two common types of cancers of the lymphatic system. The other type, non-Hodgkin's lymphoma, is far more common.

Advances in diagnosis and treatment of Hodgkin's lymphoma have helped to make this once uniformly fatal disease highly treatable, with the potential for full recovery. The prognosis continues to improve for people with Hodgkin's lymphoma.

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

Hodgkin's lymphoma signs and symptoms may include:

  • Painless swelling of lymph nodes in your neck, armpits or groin
  • Persistent fatigue
  • Fever and chills
  • Night sweats
  • Unexplained weight loss — as much as 10 percent or more of your body weight
  • Coughing, trouble breathing or chest pain
  • Loss of appetite
  • Itching
  • Increased sensitivity to the effects of alcohol or pain in your lymph nodes after drinking alcohol

When to see a doctor
These symptoms could be caused by numerous other conditions. But you should see a doctor for diagnosis if:

  • You experience symptoms for more than two weeks.
  • Your symptoms repeatedly go away and then return.

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

The exact cause of Hodgkin's lymphoma is unknown. There are two main types of Hodgkin's lymphoma: classical and nodular lymphocyte-predominant Hodgkin's lymphoma. Most people have classical Hodgkin's lymphoma, which has four subtypes:

  • Nodular sclerosing Hodgkin's lymphoma
  • Mixed cellularity Hodgkin's lymphoma
  • Lymphocyte depleted Hodgkin's lymphoma
  • Lymphocyte-rich classical Hodgkin's lymphoma

All five types are among a group of cancers called lymphomas — cancers of the lymphatic system. The lymphatic system includes the lymph nodes (lymph glands), which are located throughout your body and are connected by small vessels called lymphatics. The spleen, thymus gland and bone marrow also are part of the lymphatic system.

Hodgkin's lymphoma commonly begins in lymph nodes located in the upper part of your body. Some lymph nodes are in areas more readily noticed, such as in your neck, above your collarbone, under your arms or in your groin area. Enlarged lymph nodes in the chest cavity also are common. Eventually, Hodgkin's lymphoma may spread outside your lymph nodes to virtually any part of your body.

Abnormal B cells
A key step in Hodgkin's lymphoma involves the development of abnormal B cells. B cells are a type of lymph cell that's an important part of your immune system's response to foreign invaders. B cells normally work with T cells, which mature in the thymus, to fight infection.

When B cells develop into large abnormal cells, these abnormal, cancerous cells are called Reed-Sternberg cells. Instead of undergoing the normal cell cycle of life and death, Reed-Sternberg cells don't die, and they continue to produce abnormal B cells in a malignant process. These cells also attract other normal immune cells that cause the lymph nodes to enlarge.

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

The following are risk factors for Hodgkin's lymphoma:

  • Age. People between the ages of 15 and 40, as well as those older than 55, are most at risk of Hodgkin's lymphoma.
  • Family history. Anyone with a brother or a sister who has the disease faces an increased risk of developing Hodgkin's lymphoma, though this may be due to similar environmental exposures rather than genetic factors.
  • Sex. Males are slightly more likely to develop Hodgkin's lymphoma.
  • Past Epstein-Barr infection. People who have had illnesses caused by the Epstein-Barr virus, such as infectious mononucleosis, are more likely to develop Hodgkin's lymphoma than are people who haven't had these Epstein-Barr infections.
  • Compromised immune system. Having a compromised immune system, such as from HIV/AIDS or from having an organ transplant requiring medications to suppress your immune response, also appears to put you at a greater risk of Hodgkin's lymphoma.
  • Geography. Hodgkin's lymphoma is most prevalent in the United States, Canada and northern Europe. It's least common in Asian countries.
  • Socioeconomic status. Hodgkin's lymphoma is more common in people with higher socioeconomic background.

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

People with Hodgkin's lymphoma may be at an increased risk of the following conditions, mainly because of the effects of treatment. However, with advances in treatment, the risk of developing these complications may decline:

  • Cardiovascular disease
  • Second malignancy, such as leukemia, non-Hodgkin's lymphoma and tumors in the lungs, breasts or gastrointestinal system
  • Vascular disease, including stroke
  • Thyroid dysfunction
  • Sterility

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

If it's thought that you have Hodgkin's lymphoma, you may start by first seeing your family doctor or a general practitioner. However, in some cases when you call to set up an appointment, you may be referred immediately to a specialist who treats Hodgkin's lymphoma, such as a hematologist, medical oncologist or radiation oncologist.

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well-prepared for your appointment. Here's some information to help you get ready for your appointment, and what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet before testing.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medications, as well as any vitamins or supplements, that you're taking.
  • Take a family member or friend along, if possible. Sometimes it can be difficult to absorb all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important, in case time runs out. For Hodgkin's lymphoma, some basic questions to ask your doctor include:

  • What is likely causing my symptoms or condition?
  • Other than the most likely cause, what are other possible causes for my symptoms or condition?
  • What kinds of tests do I need?
  • Is my condition likely temporary or chronic?
  • What is the best course of action?
  • What are the alternatives to the primary approach that you're suggesting?
  • I have these other health conditions. How can I best manage them together?
  • Are there any restrictions that I need to follow?
  • Should I see a specialist? What will that cost, and will my insurance cover it?
  • Will I need a biopsy?
  • Is there a generic alternative to the medicine you're prescribing?
  • Are there any brochures or other printed material that I can take with me? What Web sites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over points you want to spend more time on. Your doctor may ask:

  • When did you first begin experiencing symptoms?
  • Have your symptoms been continuous, or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Has anyone in your family had cancer, including Hodgkin's lymphoma?
  • Have you or has anyone in your family had conditions affecting the immune system?
  • Have you had infections in the past?
  • Have you or your family been exposed to toxins?

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

Because the symptoms of Hodgkin's lymphoma are similar to those of other disorders, such as influenza, the disease can be difficult to diagnose. Some distinctive characteristics help diagnose Hodgkin's lymphoma, and these include:

  • Orderly spread to lymph nodes. The pattern of spread is orderly, progressing from one group of lymph nodes to the next.
  • Only rare 'skipping' of lymph nodes. The disease rarely skips over an area of lymph nodes as it spreads.

These tests and procedures help diagnose Hodgkin's lymphoma.

  • Biopsy. Taking a tissue sample (biopsy) of an enlarged lymph node is the most common way to make a definite diagnosis. Once a sample is removed, the tissue is examined for malignant cells.
  • Physical exam. Your doctor checks for swollen lymph nodes, including in your neck, underarm and groin, as well as a swollen spleen or liver.
  • X-rays. These pictures can reveal swollen lymph nodes in the body.
  • Computerized tomography (CT) scan. This is a series of pictures of the inside of your body, usually the chest, abdomen and pelvis. You usually swallow a dye or it's injected into your veins so that your organs and tissues will show up more clearly.
  • Magnetic resonance imaging (MRI). A device that uses a magnet, radio waves and computer provides your doctor with a series of detailed pictures of the inside of your body.
  • Positron emission tomography (PET) scan. A small amount of radioactive glucose (sugar) is injected into your vein. Glucose becomes more concentrated around cancerous cells, so when a scanner takes pictures of your body it can reveal where the cancer is located.
  • Bone marrow aspiration and biopsy. A small amount of bone marrow, blood and bone are removed through a needle and are examined for signs of cancer.
  • Blood tests. A sample of your blood is examined in a lab to see if anything in your blood indicates the possibility of cancer.

Staging Hodgkin's lymphoma
Once the diagnosis is confirmed, doctors "stage" the disease. Staging is how doctors determine the extent of the disease, which will affect your treatment options.

  • Stage I. The cancer is limited to one lymph node region or a single organ.
  • Stage II. In this stage, the cancer is in two different lymph nodes or the cancer is in a portion of tissue or an organ and nearby lymph nodes. But the cancer is still limited to a section of the body either above or below the diaphragm.
  • Stage III. When the cancer moves to lymph nodes both above and below the diaphragm, it's considered stage III. Cancer may also be in one portion of tissue or an organ near the lymph node groups or in the spleen.
  • Stage IV. This is the most advanced stage of Hodgkin's lymphoma. Cancer cells are in several portions of one or more organs and tissues. Stage IV Hodgkin's lymphoma affects not only the lymph nodes but also other parts of your body, such as the liver, lungs or bones.

Additional definitions of the cancer
Additionally, your doctor may use the letters A, B, E and S to help define the extent of your cancer and the treatment needed:

  • The letter A means that you don't have any significant symptoms as a result of the cancer.
  • The letter B indicates that you may have significant signs and symptoms, such as a persistent fever, unintended weight loss or severe night sweats.
  • The letter E stands for extranodal, which means that the cancer has spread beyond your lymph nodes.
  • The letter S designates a cancer that has spread into your spleen.

The letters B, E and S indicate potentially more serious disease.

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

The most important factor in Hodgkin's lymphoma treatment is the stage of the disease. The number and regions of lymph nodes affected and whether only one or both sides of your diaphragm are involved also are important considerations. Other factors affecting decisions about treating this disease include:

  • Your age
  • Your symptoms
  • Whether you're pregnant
  • Your overall health status

The goal of treatment is to destroy as many malignant cells as possible and bring the disease into remission. As many as 95 percent of people with stage I or stage II Hodgkin's lymphoma survive for five years or more with proper treatment. The five-year survival rate for those with widespread Hodgkin's lymphoma is about 60 to 70 percent, according to the American Cancer Society. But those numbers are based on people treated before 1990, so the outcome may be even more promising for people with more recent diagnosis and treatment.

Treatment options include:

Chemotherapy
When the disease progresses and involves more lymph nodes or other organs, chemotherapy is the preferred treatment. Chemotherapy uses specific drugs in combination to kill tumor cells. The drugs travel through your bloodstream and can reach nearly all areas of your body.

A major concern with chemotherapy is the possibility of long-term side effects and complications, such as heart damage, lung damage, liver damage, fertility problems and secondary cancers, such as leukemia.

Although severe effects aren't common, an ongoing effort is being made to find equally effective regimens with less toxicity. Drug regimens have been developed for Hodgkin's lymphoma that substantially diminish the likelihood of long-range, life-threatening complications, including acute leukemia, in people who have received multiple courses of chemotherapy and radiation therapy.

Chemotherapy regimens are commonly referred to by their initials, such as:

  • ABVD, which consists of doxorubicin (Adriamycin), bleomycin, vinblastine and dacarbazine.
  • BEACOPP, which consists of bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine and prednisone.
  • Stanford V, which consists of doxorubicin, vinblastine, mechlorethamine, etoposide, vincristine, bleomycin and prednisone. Those taking this regimen are also treated with radiation therapy.
  • COPP/ABVD, which consists of cyclophosphamide, vincristine, procarbazine, prednisone, doxorubicin, bleomycin, vinblastine and dacarbazine.
  • MOPP, which consists of mechlorethamine, vincristine, procarbazine and prednisone.

ABVD is currently the preferred treatment. Some people at high risk may receive a more intensive treatment, such as BEACOPP.

Radiation
When the disease is confined to a limited area, radiation therapy may be the treatment of choice. With radiation therapy, high-energy X-rays are used to kill cancer cells. It's typical to radiate the affected lymph nodes and the next area of nodes where the disease might progress. The length of radiation treatment varies, depending on the stage of the disease. Radiation therapy may be used alone, but it is commonly used with chemotherapy. If you relapse after radiation therapy, chemotherapy becomes necessary.

Some forms of radiation therapy may increase your risk of heart disease, stroke, thyroid problems, infertility and other forms of cancer, such as breast or lung cancer. Radiation can also damage nearby healthy tissue. Most children with Hodgkin's lymphoma are treated with chemotherapy, but they may also receive low-dose radiation therapy.

Bone marrow or stem cell transplant
If the disease returns after treatment, you may need a bone marrow or stem cell transplant. For this procedure, your own bone marrow or stem cells (autologous) are removed and treated to kill cancerous cells. Then the marrow or stem cells are frozen and stored for safekeeping. Next you receive high-dose chemotherapy to destroy cancerous cells in your body. Finally your frozen marrow or stem cells are thawed and injected into your body through your veins.

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

A diagnosis of Hodgkin's lymphoma can be extremely challenging. The following strategies and resources may make dealing with cancer easier:

  • Know what to expect. Find out everything you can about your cancer — the type, the stage, your treatment options and their side effects. The more you know, the more active you can be in your own care. In addition to talking with your doctor, look for information in your local library and on the Internet. The National Cancer Institute answers questions from the public and can be reached at 800-422-6237. Or you can contact the American Cancer Society at 800-227-2345.
  • Be proactive. Although you may feel tired and discouraged, don't let others — including your family or your doctor — make important decisions for you. Take an active role in your treatment.
  • Maintain a strong support system. Having a support system and a positive attitude can help you cope with any issues, pain and anxieties that might occur. Although friends and family can be your best allies, they sometimes may have trouble dealing with your illness. If so, the concern and understanding of a formal support group or others coping with cancer can be especially helpful.

Although support groups aren't for everyone, they can be a good source for practical information. You may also find you develop deep and lasting bonds with people who are going through the same things you are.

  • Set reasonable goals. Having goals helps you feel in control and can give you a sense of purpose. But don't choose goals you can't possibly reach. You may not be able work a 40-hour week, for example, but you may be able to work at least part time. In fact, many people find that continuing to work can be helpful.
  • Take time for yourself. Eating well, relaxing and getting enough rest can help combat the stress and fatigue of cancer. Also, plan for the downtimes when you may need to rest more or limit what you do.
  • Stay active. Receiving a diagnosis of cancer doesn't mean you have to stop doing the things you enjoy or normally do. For the most part, if you feel well enough to do something, go ahead and do it. It's important to stay active and involved as much as you can.

©1998-2009 Mayo Foundation for Medical Education and Research (MFMER). Terms of use.

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