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Hodgkin’s disease is a lymphoma, a cancer of the lymphatic system. Hodgkin’s disease and non-Hodgkin’s lymphoma are the two types of lymphomas. Hodgkin’s disease is distinguished by the presence of large abnormal cells, called Reed-Sternberg cells. The disease is less common than non-Hodgkin’s lymphoma.
Hodgkin’s disease is classified into two main types:
Hodgkin’s disease is considered one of the most curable forms of cancer, especially if it is diagnosed and treated early. Five-year survival rates for patients diagnosed with stage I or stage II Hodgkin’s disease are 90 - 95%. Many patients with late-stage Hodgkin’s disease also have good odds for survival.
Hodgkin's disease occurs most often in people aged 15 - 40 (especially in their 20s), and in people over age 55. About 10 - 15% of Hodgkin’s disease cases are diagnosed in children and teenagers. It is slightly more common in males than in females.
Certain types of viral infections may increase the risk of Hodgkin’s disease. Infectious mononucleosis, which is caused by the Epstein-Barr virus, is associated with increased risk as is infection with the human immunodeficiency virus (HIV).
Chemotherapy and radiation are the main treatments for Hodgkin’s disease. Patients who have relapsed may be treated with autologous stem cell transplantation, a procedure which uses the patient's own blood cells.
Preventing Infection after Cancer Treatment
Both chemotherapy and stem cell transplants increase the risk for serious infections. Patients must take precautions to avoid exposure to germs. Ways to prevent infection include:
Hodgkin's disease is a type of lymphoma. Lymphomas are cancers of the lymphatic system. They are generally subdivided into two groups: Hodgkin's disease (HD) and non-Hodgkin's lymphoma (NHL). Hodgkin’s disease is also called Hodgkin’s lymphoma.
Hodgkin’s disease is marked by the presence of abnormal large cells called Reed-Sternberg cells. Reed-Sternberg cells are derived from B cell lymphocytes (white blood cells). Reed-Sternberg cells are specific to Hodgkin’s disease. They are not found in non-Hodgkin’s lymphoma.
HD usually starts in B cell lymphocytes located in lymph nodes in the neck area, although any lymph node may be the site of initial disease.
There are two main types of Hodgkin’s disease:
Classical Hodgkin's Lymphoma. Classical Hodgkin’s lymphoma accounts for about 95% of Hodgkin’s disease cases. It has four major subtypes:
Nodular Lymphocyte-Predominant Hodgkin's Disease. Nodular lymphocyte-predominant Hodgkin's disease occurs in about 5% of patients. It is distinct from classical Hodgkin’s lymphoma. The cells look like and are referred to as “popcorn” cells, which are variants of Reed-Sternberg cells. This type of HD typically affects younger patients and usually originates in the neck lymph nodes. It is sometimes confused with non-Hodgkin’s lymphoma (NHL). In fact, there is a 3 - 5% risk that nodular lymphocyte-predominant Hodgkin’s disease can transform into diffuse large B-cell NHL.
Lymphomas are tumors of the lymphatic system. This system is a network of organs, ducts, and nodes. The lymphatic system transports a watery clear fluid called lymph throughout the body. The lymphatic system contains lymphocytes, which are important cells involved in defending the body against infections.
Lymphocytes. Lymphocytes are a type of white blood cell. They are an essential part of the immune system:
Lymphatic vessels. Lymphatic vessels begin as tiny tubes. These tubes collect and carry fluids that leak from body tissues, lymphocytes, proteins, and other substances collected from the body's tissues. The tubes lead to larger lymphatic ducts and branches, which drain into two ducts in the neck, where the fluid re-enters the bloodstream.
Lymph Nodes. Along the way, the fluid passes through lymph nodes, which are oval structures made up of lymph vessels, connective tissue, and white blood cells:
Other Structures in the Lymphatic System. The tonsils and adenoids are secondary lymphatic organs. They are composed of masses of lymph tissue that also play a role in the lymphatic system. The spleen is another important organ that processes lymphocytes from incoming blood.
Spread of Cancer. Tumor cells can enter the lymph fluid and travel to the lymph nodes. Hodgkin's disease usually progresses in an orderly way from one lymph node region to the next. This process may be slow, particularly in younger people, or very rapid. The disease typically spreads downward from the initial site:
Hodgkin’s disease is less common than non-Hodgkin’s lymphoma. It accounts for about 10% of all lymphomas. According to the American Cancer Society, about 9,000 new cases of Hodgkin's disease (HD) are diagnosed in the United States each year. The exact causes of Hodgkin’s disease are unknown. Research indicates that the malignant process leading to Hodgkin's disease may be triggered by a combination of environmental and genetic factors along with a susceptible immune system.
Hodgkin's disease occurs most often in people ages 15 - 40 (especially in their 20s), and in people over age 55. About 10 - 15% of Hodgkin’s disease cases are diagnosed in children and teenagers.
Hodgkin's disease is slightly more common among males than females. Women who get Hodgkin's disease appear to have a slightly lower risk for relapse after treatment than men.
Infectious mononucleosis (“mono”), which is caused by the Epstein-Barr virus (EBV), is linked with increased risk for Hodgkin’s disease. However, only 1 in 1,000 patients with mononucleosis develops Hodgkin's disease. The Epstein-Barr virus is present in 90% of all people and, in the great majority of these cases, the virus causes a mild case of mononucleosis or no illness at all. Only a very small percentage of people who have had mononucleosis go on to develop HD. Other factors must be present to trigger the malignancy.
People infected with the human immunodeficiency virus (HIV), which weakens the immune system, are also at increased risk of developing Hodgkin’s disease.
Hodgkin's disease runs in families in about 5% of cases. Siblings of patients have a three times higher risk than the general population.
Symptoms of Hodgkin’s disease may include:
The last three symptoms (weight loss, fever, and night sweats) are classified as “B symptoms.” B symptoms may be used in staging Hodgkin’s disease and can indicate that more aggressive treatment will be required.
Sometimes patients with Hodgkin’s disease do not experience any symptoms, or symptoms may not appear until the cancer is very advanced. Enlarged lymph nodes can also be caused by many noncancerous conditions, such as infections.
The doctor will take a medical history and perform a physical examination. If these procedures indicate Hodgkin's disease, a number of additional tests may be needed to either rule out other diseases or confirm HD and determine the extent of the cancer.
The doctor will examine not only the affected lymph nodes but also the surrounding tissues and other lymph node areas for signs of infection, skin injuries, or tumors. The consistency of the node is evaluated. For example, a stony, hard node is often a sign of cancer, usually one that has metastasized (spread to another part of the body). A firm, rubbery node may indicate lymphoma (including Hodgkin's). Soft tender nodes suggest infection or inflammatory conditions.
Blood tests are performed to measure white and red blood cells, blood protein levels, the uric acid level, blood proteins, and the liver's function.
Chest X-Ray. A chest x-ray may show lymph nodes in the chest, where Hodgkin's disease usually starts. It is a useful step for detecting enlarged lymph nodes.
Computed Tomography. Computed tomography (CT) scans are much more accurate than x-rays. They can detect abnormalities in the chest and neck area, as well as revealing the extent of the cancer and whether it has spread. CT scans are used to evaluate symptoms and help diagnose lymphomas, help with staging of the disease, and monitor response to treatment. A CT scan is also often used to detect lymphomas in the abdominal and pelvic areas, the brain, and chest area.
Positron Emission Tomography (PET). PET scans combined with CT scans can help doctors clarify the location of the cancer. PET scans can also provide information on whether or not an enlarged lymph node is benign or cancerous and can be used for staging lymphomas. PET scans may also help doctors determine how well a patient has responded to treatment, if any residual cancer exists, and if a patient has achieved remission.
A biopsy of the suspicious lymph node is the definitive way to diagnose Hodgkin's disease. The lymph node sample will be examined by a pathologist for the presence of Reed-Sternberg cells or other abnormal features.
The type of biopsy performed depends in part on the location and accessibility of the lymph node. The doctor may surgically remove the entire lymph node (excisional biopsy) or a small part of it (incisional biopsy). In some cases, the doctor may use fine needle aspiration to withdraw a small amount of tissue from the lymph node. Biopsies of bone marrow may also be performed in patients with existing Hodgkin's disease if the doctor suspects that it may have spread to the marrow.
Hodgkin’s disease is considered one of the most curable forms of cancer, especially if it is diagnosed and treated early. Unlike other cancers, Hodgkin's disease is even potentially curable in late stages.
A 5-year survival rate is the percentage of patients who live at least 5 years after their cancer is first diagnosed. Five-year survival rates for patients diagnosed with stage I or stage II Hodgkin’s disease are about 90%. With advances in treatment, even most patients diagnosed with advanced Hodgkin’s disease live longer than 5 years.
Outlook tends to be poorer for patients who do not respond to first-line therapy or who relapse within a year of treatment. Patients who survive 15 years after treatment are more likely to later die from other causes than from Hodgkin’s disease.
Survival rates are a general term based on data collected from large numbers of people. A patient’s prognosis depends on factors specific to that individual. Factors that influence prognosis and survival include age, overall health, stage of cancer at time of diagnosis, symptoms associated with the cancer, and how well the cancer responds to treatment.
The International Prognostic Factors Project on Advanced Hodgkin's Disease uses seven factors to help determine which patients with advanced Hodgkin's disease have a more serious prognosis and could benefit from more aggressive chemotherapy. These factors are also used to predict success in patients with relapsed or persistent HD who are undergoing stem cell transplantation.
The more of these factors that are present, the worse the outlook and the more likely the patient needs to be treated aggressively:
The good news about Hodgkin's disease is that treatment can cure the disease. The bad news is that survivors face a higher than average risk for long-term complications of these treatments, some very serious.
Many patients experience chronic fatigue that can sometimes last for years. The most serious complications are secondary cancers and heart disease, which may develop over the 20 - 30 years following treatments.
Secondary cancers include non-Hodgkin's lymphoma, leukemia, melanoma, stomach and lung cancers, and breast and uterine cancers. Heart disease complications include coronary artery disease, stroke, heart valve problems, and cardiomyopathy (weakening of the heart muscle). Thyroid disorders are also a potential complication. Combinations of radiation and chemotherapies pose the highest risk of these problems.
Studies of adult survivors of various childhood cancers have found that 30 years after treatment, patients with cured Hodgkin’s disease are especially likely to have other serious health problems. Female survivors face a significantly greater risk than male survivors. In particular, women who received chest radiation are at very high risk for developing breast cancer.
Patients with Hodgkin’s disease should get a written record of the treatments they received as children, and the potential risks of these treatments. These records can help the doctors who later oversee their care monitor for potential health problems.
Survivors of Hodgkin’s disease should receive regular screening tests for cancer and heart disease. They may need to get these tests at a younger age than most patients. In particular, patients who were treated with chest radiation should get blood tests every 5 years to measure their cholesterol levels. Female patients who received chest radiation should get early and frequent mammograms.
Treatment options for Hodgkin’s disease depend on:
Certain factors may determine whether more intensive treatment is required. For example, the presence of B symptoms and “bulky” (large mass) tumors usually indicates a more aggressive treatment approach.
Chemotherapy and radiation are the main treatments for Hodgkin’s disease. Stem cell transplantation or a biologic drug may be recommended for patients whose cancer has recurred.
Hodgkin’s disease is staged (I through IV) depending on how far the cancer has spread. Staging is the primary method for determining both treatment options and prognosis.
Stage I. Disease is limited to a single node region (I) or has involved one neighboring area or a single nearby organ.
Stage II. Disease is limited to two or more lymph nodes on the same side of (above or below) the diaphragm or extends locally from the lymph node into a nearby organ.
Stage III. Disease is in lymph nodes on both sides of the diaphragm or has spread to nearby organs, the spleen, or both.
Stage IV. Disease has become widespread involving organs outside the lymph system, such as liver, lung, or bone marrow.
Early Stages (I or II). Hodgkin’s disease in stages I or II is usually treated with chemotherapy followed by radiation.
Later Stages (III and IV). Hodgkin’s disease in stages III and IV is usually treated with chemotherapy alone. .
Refractory and Relapsed Hodgkin's. Treatment is considered successful when the signs and symptoms of cancer disappear. This is referred to as remission. Cancer that does not respond to treatment is called refractory or resistant. Cancer that recurs after remission is called relapsed. Treatments for refractory or relapsed Hodgkin’s include high dose chemotherapy with stem cell transplantation. Patients who are not good candidates for transplantation or who have not been helped by it may benefit from treatment with the biologic drug brentuximab (Adcetris).
Preventing Infection. Both the disease and some of the treatments suppress the immune system, increasing the risk for infections. Widespread, life-threatening infection is a particular danger if the spleen has been removed and both radiation and chemotherapy are administered. Patients should be vaccinated against pneumococcus, meningococcus, and Haemophilus influenza bacteria before receiving treatment.
Preserving Fertility. Patients who may wish to have children in the future should ask their doctors about fertility-preserving treatments. It is very important to have these discussions before cancer treatment starts. The American Society for Clinical Oncology (ASCO) has guidelines for the best fertility preservation methods for male and female cancer patients. For men, ASCO recommends banking and freezing sperm (sperm cryopreservation) for later use in assisted reproductive therapies.
For women, egg (oocyte) cryopreservation is recommended. This procedure involves harvesting and freezing a woman's eggs (oocytes), and can be followed by in vitro fertilization and freezing of embryos for later use. It requires several weeks of pre-treatment with ovarian stimulation drugs, so timing is very important. For women who will receive radiation therapy to the pelvic region, a surgical procedure that moves the ovaries out of the path of radiation (ovarian transposition) can also help preserve fertility.
Relapse of Hodgkin’s disease is not uncommon, even after treatment for early stages. It can occur a decade or more after treatment. Relapse is more likely to occur in early-stage disease, probably because limited radiation normally used in such cases does not destroy all malignancies. Patients who had large tumors in the chest are also at higher risk for recurrence.
Patients need periodic examinations and imaging tests for years after treatment, both to check for signs of relapse as well as to monitor the long-term effects of treatments.
Because Hodgkin's disease often occurs in young adults, treatment for pregnant women is of particular concern. Therapy must be effective enough to protect the mother without hurting the fetus. Chemotherapy is rarely used early in the term, because it poses a risk for birth defects.
Treatment choice must be individualized, taking into consideration the mother's wishes, the severity and pace of the disease, and the remaining length of the pregnancy. The treatment plan may need to be changed as the pregnancy progresses. If the disease develops in the second half of the pregnancy, it may be possible to postpone chemotherapy or radiation therapy until after an early induced delivery.
Chemotherapy is usually the first treatment for all stages of Hodgkin’s disease. Chemotherapy uses drugs to kill cancer cells. The drugs are called cytotoxic (“toxic to cells”) medications. Chemotherapy is referred to as body-wide, or systemic, therapy because the drugs affect cells throughout the body.
Chemotherapy drugs may be taken by mouth as pills or given by injection or infusion. Treatment may be administered at a medical center, doctor's office, or even a patient's home. Some patients receiving chemotherapy may need to remain in the hospital for several days so the effects of the drug can be monitored.
Patients typically receive 4 - 8 cycles of chemotherapy, depending on the stage. A cycle is usually 28 days and consists of several doses of drug administration followed by a period of rest.
Several chemotherapy regimens are used for treating Hodgkin’s disease. Standard regimens include ABVD, Stanford V, and BEACOPP.
ABVD is used to treat adults and children in both early and late stages of Hodgkin’s disease. For early stages (I and II), patients typically receive 2 cycles of the drugs, followed by radiation. In late stages (III and IV), patients receive 6 – 8 cycles of chemotherapy. ABVD consists of a 4-drug combination:
Stanford V is usually reserved for late-stage Hodgkin’s disease. It consists of a 7-drug combination:
BEACOPP is a chemotherapy regimen reserved for late-stage Hodgkin’s disease. It is effective but can increase risk for developing secondary cancers such as leukemia. Patients who are treated with BEACOPP should receive long-term follow-up care to monitor for side effects from this therapy. BEACOPP consists of 7 drugs:
Brentuximab (Adcetris) is a newer biologic drug that is used by itself. It is approved for patients with Hodgkin’s disease who have either:
Brentuximab works by targeting CD-30, a protein found on Hodgkin’s cancer cells. The drug is given by intravenous infusion. The most common side effects are neutropenia, peripheral sensory neuropathy, fatigue, nausea, and anemia. A more serious but rare side effect is the brain disorder progressive multifocal leukoencephalopathy (PML).
Side effects and complications of any chemotherapeutic regimen are common, are more severe with higher doses, and increase over the course of treatment.
Common Side Effects. Common side effects of chemotherapy include:
Serious Side Effects. Serious side effects can also occur and may vary depending on the specific drugs used. They include:
Long-Term Complications. Some side effects of chemotherapy may linger after treatment or may develop long after the treatment has ended. Be sure to discuss with your doctor what tests you may need to monitor the long-term effects of chemotherapy treatment. Long-term complications of chemotherapy for Hodgkin’s disease may include:
Radiation therapy, which shrinks tumors, used to be the main treatment for Hodgkin's disease. Today, radiation therapy is mainly used to treat early stage (I or II) Hodgkin’s disease and is usually given following chemotherapy.
Involved field radiation is the preferred method of radiation therapy for treating Hodgkin’s disease. It targets only the lymph node regions that are known to have cancer, not the adjacent, uninvolved lymph node regions. Involved-field radiation is usually given after several rounds of chemotherapy.
Involved field radiation is a type of external-beam radiation therapy. The patient lies on a table while a machine delivers high-energy x-rays to specific targets on the body.
Extended field radiation, an older approach that targeted both the diseased lymph nodes as well as surrounding healthy lymph nodes, is no longer used.
Doctors are working on refining radiation therapies for Hodgkin's disease so that they more precisely target the affected lymph nodes and deliver the lowest possible effective dose of radiation. The aim is to destroy the cancerous cells while minimizing the damage to healthy cells and causing fewer side effects.
Newer radiation techniques for Hodgkin’s disease include involved node radiation therapy, involved site radiation therapy, intensity modulated radiation therapy, and proton therapy.
Fatigue, nausea, diarrhea, dry mouth, skin irritation, and increased risk for infections are common short-term side effects of radiation therapy. These side effects generally clear up after treatment is completed.
Radiation therapy can cause more serious long-term complications, which is why researchers are working on techniques that can reduce the radiation doses and increase the accuracy of the beams. These side effects generally depend on the radiation target site in the body. They include:
This treatment involves removal and replacement of stem cells, which are produced in the bone marrow. This allows the patient to receive high-dose chemotherapy without destroying these important cells. Stem cells are the early forms for all blood cells in the body (including red, white, and immune cells). Cancer treatments harm growing cells as well as cancer cells, and so the healthy stem cells must be replaced by transplanting them.
For Hodgkin’s disease, the most common type of transplant is an autologous procedure, using the patient’s own cells. An allogeneic transplant, using cells from a donor, is more risky for patients with Hodgkin’s disease and is generally used only when an autologous transplant has failed. Allogeneic transplants are sometimes used as treatments for non-Hodgkin’s lymphoma. They have a greater risk than autologous transplants for complications such as graft-versus-host disease.
Stem cells must first be collected in one of the following ways:
Stem cells are collected several weeks before the procedure. They are frozen and stored while the patient undergoes high-dose chemotherapy. Some patients receive high-dose whole body radiation therapy along with chemotherapy.
After the patient completes the pre-transplant therapy, the frozen cells are thawed and then infused into the patient. Within a few weeks, these cells start to generate new white blood cells and then new red blood cells.
The risk for infection is greatest during the first 6 weeks following the transplant. During this period, a patient usually remains in isolation and receives antibiotics and intravenous nutrition. It takes 6 - 12 months post-transplant for a patient’s immune system to fully recover.
Many patients develop severe herpes zoster virus infections (shingles) or have a recurrence of herpes simplex virus infections (cold sores and genital herpes). Pneumonia, cytomegalovirus, aspergillus (a type of fungus), and Pneumocystis jiroveci (a fungus) are among the most serious life-threatening infections.
It is very important that patients take precautions to avoid infections. Guidelines for infection prevention include:
Early side effects of transplantation are similar to chemotherapy and include nausea, vomiting, fatigue, mouth sores, and loss of appetite. Bleeding because of reduced platelets is a high risk during the first four weeks and may require transfusions. Later side effects may include fertility problems (if the ovaries are affected), thyroid gland problems (which can affect metabolism), lung damage (which can cause breathing problems), other organ damage, and bone damage.
In younger patients, there is a small long-term risk for leukemia after transplantation. Chemotherapy itself increases the risk of secondary cancers. Recent studies suggest that transplantation after chemotherapy does not add any additional risks.
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