Hodgkin lymphoma occurs when cells within the lymph system begin to grow out of control. The abnormal cells form tumors and can also spread to other parts of the body. There are lymph nodes clustered in groups all over our bodies, and Hodgkin lymphoma can begin in any of those nodes, but it most commonly starts in the nodes in the neck, groin or underarm. Hodgkin lymphoma is distinguished from other types of lymphomas by the way the cells look under the microscope and the way it grows and spreads. One feature of Hodgkin lymphoma is the presence of Reed-Sternberg cells, large, cancerous cells that are not normally found in lymphatic tissue.
Hodgkin lymphoma occurs in both adults and children. These are discussed separately.
Perspective: Swollen lymph nodes (or glands). Just about everyone has had a swollen lymph node at sometime during their life. These grape sized glands are a key part of our immune system. When an infection occurs, they react, often becoming enlarged and/or sore in the process. Most swollen lymph glands return to normal size on their own within a week of two as the infection subsides. A lymph node that is enlarged, painless and does not go away should be examined by a doctor.
There are approximately 9,000 new cases of Hodgkin lymphoma diagnosed in the United States every year, accounting for 11.7 of all lymphomas. This type of cancer often affects people in two age groups: adolescents and young adults, and those between the ages of 60-84. It occurs somewhat more often in boys and men than in women. Advances in treatment have made Hodgkin lymphoma one of the most treatable forms of cancer. Today over 80% of patients are cured of their disease.
The causes of Hodgkin lymphoma are not known. Possible risk factors include:
The early stages of Hodgkin lymphoma do not cause any symptoms. The most common sign is a painless, enlarged lymph node, usually in the neck, groin or underarm. Sometimes the patient notices this swollen node; others are discovered during routine physical examinations.
As it advances, Hodgkin lymphoma can cause a number of symptoms, including:
If the doctor suspects Hodgkin lymphoma, the usual next step is to remove and biopsy an enlarged lymph node. The entire node is removed so that the pathologist can examine it under the microscope. If there is no enlarged node, but the symptoms suggest Hodgkin’s lymphoma, then other tests are necessary, but the diagnosis always requires tissue from a biopsy.
The pathologist’s job is to determine whether there is cancer present in the lymph node, and if so exactly what kind of lymphoma it is. All Hodgkin lymphomas are characterized by the presence of abnormal cells called Reed-Sternberg cells which distinguish them from other types of lymphoma. There are two main types: classical Hodgkin lymphoma which accounts for 95% of all cases and lymphocyte depleted Hodgkin lymphoma. Knowing the type of Hodgkin lymphoma is important to deciding the treatment.
Cancer is defined as a group of more than 100 different diseases, but there are many subtypes of specific cancers as well. In recent years, research has made it possible to identify more and more of these subtypes and to understand how these differences influence the cancer’s behavior and its treatment. Four subtypes of classical Hodgkin lymphoma have been defined, each identified by the kinds of cells that the pathologists sees under the microscope. These are:
Nodular Sclerosis Hodgkin lymphoma.
This accounts for 80% of classical Hodgkin lymphoma. It is most common in young adults. In addition to Reed Sternberg cells, there are bands of connective tissue in the lymph node. This kind of Hodgkin lymphoma is often associated with the involvement of lymph nodes in the neck and/or chest.
Lymphocyte rich Hodgkin lymphoma.
About 6% of patients have this type. It is more common in men. The tissue contains many more normal blood cells called lymphocytes in addition to Reed Sternberg cells.
Mixed cellularity Hodgkin Lymphoma.
This occurs more often in older adults and is often found in the abdomen. It has many different kinds of cells.
Lymphocyte depleted Hodgkin lymphoma.
This is the least common type of Hodgkin lymphoma, accounting for about 1% of patients. It is more common in older adults or people with HIV or AIDs infection. The lymph nodes contain almost all Reed Sternberg cells.
Nodular lymphocyte predominant Hodgkin lymphoma (NLPHD).
This type of Hodgkin lymphoma is usually very slow growing and is sometimes treated with different chemotherapy than other forms of Hodgkin lymphoma.
It is easy to get lost in the complex terms that are used to describe these subtypes. Depending on how you approach your disease—and how much information you want—you may choose to learn the details of your specific subtype. Or, you may choose to understand these subtypes in broader terms—what they mean to your treatment and prognosis.
If the biopsy is positive and the diagnosis is Hodgkin lymphoma, a patient usually undergoes a series of tests to determine the extent of the cancer. These can include:
Blood and lab tests.
These are done to analyze the different types of blood cells. Liver function tests are also standard as are tests to measure the ESR, or erythrocyte sedimentation rate which can be a prognostic indicator.
CT , MRI or PET scan.
Scans are done to determine if the Hodgkin lymphoma has spread to other groups of lymph nodes or organs in the body.
Bone marrow biopsy and aspiration.
Lymphoma often spreads to bone marrow, the spongy tissue inside of bones. A bone marrow biopsy uses a needle to remove a small amount of solid tissue from the marrow, while the aspiration withdraws a sample of the fluid portion of the bone marrow. The most common site for a bone marrow biopsy is the pelvic bone located in the lower part of the back. This is done as an outpatient procedure.
Staging means using all the information available about a cancer to describe its location and the extent to which it has spread. In staging Hodgkin lymphoma, doctors evaluate the following:
Every Hodgkin lymphoma is staged from I to IV, with I being the least extensive and IV the most. In addition, the letters A is used if a patient does not have certain symptoms, and B if a patient has experienced these symptoms, including weight loss, fever, and drenching night sweats.
Stage I: The cancer if found in only one lymph node region
Stage II: Either, --The cancer is in two or more lymph node regions on the same side of the diaphragm, or --The cancer involves a single organ and its regional lymph nodes—those located near the organ—with or without cancer in other lymph nodes on the same side of the diaphragm. (IIE)
Stage III: The cancer involves lymph nodes on both sides of the diaphragm. In addition, there can be involvement in another organ (IIIE), involvement of the spleen (IIIS), or both (IIIES).
Stage IV: The Hodgkin lymphoma has spread to multiple organs or lymph node areas. These can include the bone marrow, liver, or lungs.
Staging is very important to deciding on the best treatment option for each patient. In addition, there are other risk factors that influence the prognosis for Hodgkin lymphoma. Age. Younger patients often have a better prognosis than older patients.
Gender: Women have a better prognosis than men.
Subtype: People with lymphocyte predominant, nodular sclerosing and lymphocyte rich Hodgkin lymphoma, and nodular lymphomcyte predominant type have better prognoses than other subtypes.
Having a large mass or solid tumor (more than 10cms) in the center of the chest, an area known as the mediastinum is a risk factor.
Having certain symptoms such as weight loss, night sweats or fever is a risk factor. Having a higher ESR (erythrocyte sedimentation rate) is a risk factor Blood chemistry—a low albumin is a risk factor
Blood count—all of these are risk factors
If you have been diagnosed with Hodgkin lymphoma, your doctors will look at all the characteristics of your cancer, the type, stage and other prognostic factors and assign your cancer to one of three treatment groups.
Early favorable: These are stage I and II Hodgkin lymphomas without other factors that have a negative influence on the prognosis
Early Unfavorable: These are stage I and II Hodgkin lymphomas in which there are one or more risk factors that make the prognosis less favorable.
Advanced Favorable: Stage III or IV disease with three or fewer of the risk factors that influence prognosis
Advanced Unfavorable: Stage III or IV disease with four or more of these risk factors
Perspective: These groups have helped doctors deliver effective treatment to Hodgkin lymphoma patients while minimizing the side effects, especially the long term complications that are caused by the treatment rather than the disease. The early treatments for Hodgkin lymphoma developed during the 1960s and 70s represented an important advance in cancer treatment, but often resulted in problems such as infertility and second cancers later in life. A major goal of current therapy is to cure the cancer, or manage it effectively with as few complications as possible.
The treatment for Hodgkin lymphoma is often multidisciplinary, meaning that it involves different doctors using different approaches. These include chemotherapy and radiation therapy, often in combination with each other. The exact treatment is guided by the stage and other prognostic factors. Patients with Hodgkin lymphoma should be treated in a center that can offer a multidisciplinary approach to both the medical treatment and the supportive care of patients, and that has experience and expertise in treating this disease. The team can include:
Most patients with early (stage I and II) favorable disease will receive a combination of chemotherapy and radiation therapy to the lymph node areas affected by the cancer. There are several drug combinations that are used for Hodgkin lymphoma, all of which are potentially very effective in controlling early disease. The optimal treatment will vary according to the individual’s type and stage, but the goal is to achieve a permanent, total remission with the fewest possible side effects and complications. For some carefully selected early Hodgkin lymphoma patients, it may be possible to treat the cancer with a short course of chemotherapy and no additional radiation therapy. Other patients may require radiation therapy to the affected areas of the body without chemotherapy. There are many clinical trials underway today comparing these different combinations and determine their best uses.
Patients with risk factors that make their cancers more likely to recur receive combination chemotherapy often with radiation therapy to the affected parts of their body.
More Info: Hodgkin lymphoma can affect pregnant women. When this happens, every effort is made to spare the fetus. This may mean choosing active surveillance—watching and monitoring the cancer without treating it until after the pregnancy. These patients may also receive chemotherapy agents that have not been linked to birth defects or known to harm the fetus or undergo therapy with steroids. Cancer in pregnancy is a very difficult and complicated situation and should be managed by a team of doctors and support staff who are very familiar with the medical and psychological issues involved.
For patients with stage III and IV disease, chemotherapy is the primary treatment. Some patients may also undergo radiation therapy to the affected areas. This stage of Hodgkin lymphoma often requires a more aggressive approach to treatment. This can involve combinations of chemotherapy drugs, or chemotherapy with a stem cell transplant (see below). Patients with advanced unfavorable Hodgkin lymphoma should also consider participating in clinical trials which study new drugs or treatment approaches. There are a number of open trials for patients with this kind of Hodgkin lymphoma. For more information about clinical trials and how they work, click here (link).
Hodgkin lymphoma patients who are treated with chemotherapy and radiation but whose disease is not cured, and some patients with advanced unfavorable disease may be candidates for a stem cell transplant. For a complete description of how stem cell transplants work, go to link. Stem cell transplants sometimes result in permanent remissions for patients who have not responded to other treatments--but this is a complicated procedure which involves intense treatment and risks to the patient.
Once a person has finished active treatment for Hodgkin lymphoma, he or she will need frequent follow up examinations to check for recurrence. At first, these checkups will be every few months and involve physical examination and, lab tests. CT or PET scans may be used if indicated by the examination, blood work, or symptoms.. The treatments for Hodgkin lymphoma can cause both short and long term side effects, including heart, lung and thyroid problems, fertility issues and the risk of developing a second cancer. All of these are important. For this reason, follow up also involves surveillance for these problems as well. People who have been treated for Hodgkin lymphoma often benefit from counseling or from participating in a support group.
It is a good idea to talk to your treatment team about your follow up care and to work with them on a written treatment plan. This is a very useful way of making sure that you and everyone involved in your care understands what needs to be done and the frequency of your follow visits.