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INTRODUCTION — Hodgkin lymphoma (formerly called Hodgkin's disease) is a cancer of the body's lymphatic system. Lymphomas are cancers of lymphocytes, a type of white blood cell that is important in the immune system.
The lymphatic system is a network of lymph nodes and interconnecting lymph vessels (figure 1). Lymph nodes are small, bean-shaped organs that make and store lymphocytes, a type of white blood cell that fights infection. Lymph vessels are similar to blood vessels and carry a watery fluid (lymphatic fluid) that contains lymphocytes.
In Hodgkin lymphoma, a cancerous tumor develops in a lymph node, usually in the neck or chest. If Hodgkin lymphoma spreads, it usually spreads first to nearby lymph nodes, and then to the spleen, liver, or bone marrow. As it progresses, Hodgkin lymphoma can affect the body's ability to fight infection. The exact cause of Hodgkin lymphoma is not known in most cases.
Fortunately, Hodgkin lymphoma is one of the most treatable forms of cancer. More than 75 percent of people diagnosed with Hodgkin lymphoma can be cured with the initial treatment. Over 90 percent of people live at least 10 years after treatment.
More detailed information about Hodgkin lymphoma is available by subscription. (See "Pretreatment evaluation, staging, and treatment stratification of classic Hodgkin lymphoma" and "Treatment of favorable prognosis early (stage I-II) classic Hodgkin lymphoma" and "Treatment of unfavorable prognosis early (stage I-II) classic Hodgkin lymphoma in adults" and "Initial treatment of advanced (stage III-IV) classic Hodgkin lymphoma".)
CANCER CARE DURING THE COVID-19 PANDEMIC — COVID-19 stands for "coronavirus disease 2019." It is an infection caused by a virus called SARS-CoV-2. The virus first appeared in late 2019 and has since spread throughout the world. Getting vaccinated lowers the risk of severe illness; experts recommend COVID-19 vaccination for anyone with cancer or a history of cancer.
In some cases, if you live in an area with a lot of cases of COVID-19, your doctor might suggest rescheduling or delaying medical appointments. But this decision must be balanced against the importance of getting care to screen for, monitor, and treat cancer. Your doctor can talk to you about whether to make any changes to your appointment schedule. They can also advise you on what to do if you test positive or were exposed to the virus.
HODGKIN LYMPHOMA SYMPTOMS — Most people with Hodgkin lymphoma are diagnosed because of a painless, enlarged lymph node in the neck. Enlarged nodes may also be found above the collar bone, in the armpit (axilla), or the groin (inguinal) area (figure 1).
Some people are diagnosed when a mass in the chest causes a cough, chest discomfort, or shortness of breath. There may also be symptoms of fever, night sweats, weight loss, intensive itchiness, or pain in a lymph after consuming alcohol. (See "Clinical presentation and diagnosis of classic Hodgkin lymphoma in adults".)
If your doctor or nurse is concerned that you could have Hodgkin lymphoma, you should see a physician who specializes in cancer treatment (called a hematologist/oncologist) for further testing.
HODGKIN LYMPHOMA DIAGNOSIS
Tissue biopsy — If you have symptoms of Hodgkin lymphoma, you will need surgery to remove an enlarged lymph node or core needle biopsies; both approaches are done as day surgery procedures. After removal, the lymph node is examined to see whether it contains any signs of lymphoma. (See "Clinical presentation and diagnosis of classic Hodgkin lymphoma in adults".)
HODGKIN LYMPHOMA STAGING — Staging involves dividing people with Hodgkin lymphoma into groups (stages) based on certain criteria at the time of diagnosis. Treatment decisions are based in large part on the stage of disease that is found. (See "Pretreatment evaluation, staging, and treatment stratification of classic Hodgkin lymphoma".)
The following are terms used in the staging criteria:
●Lymph node regions: An area of lymph nodes and the surrounding tissue. Examples include the cervical nodes in the neck, the axillary nodes in the armpit, the inguinal nodes in the groin, or the mediastinal nodes in the chest (figure 1).
●Lymph structures: Organs or structures that are part of the lymphatic system, such as the lymph nodes, spleen, and thymus gland (figure 1). These organs or structures play a role in the body's immune system.
●Diaphragm: A large muscle that separates the chest from the abdomen.
The stage of Hodgkin lymphoma is based on:
●The number of lymph node regions or structures that are involved
●The location of the affected lymph node regions or structure (one or both sides of the diaphragm)
●Whether there are signs of cancer outside the lymphatic system (eg, in the liver, lung, or bone marrow)
●Whether you have unexplained fever, night sweats, or weight loss (called "B symptoms")
Hodgkin lymphoma stages range from stage I (only one lymph node region or structure is involved) to stage IV (the cancer has spread beyond the lymphatic system) (table 1).
In general, lower-stage cancers are more likely to be cured and less likely to come back after treatment compared with higher-stage cancers. Stage I and II Hodgkin lymphoma are referred to as early stage, while stage III and IV Hodgkin lymphoma are referred to as advanced.
Subclassifications — Additional criteria help to further identify subgroups within each stage, as follows:
●A or B – The letter "A," as in stage IIA, means that symptoms of unexplained fever, night sweats, or weight loss (at least 10 percent of the body weight) were not present during the six months prior to diagnosis. The letter "B," as in stage IIIB, means that these symptoms were present. These symptoms are therefore referred to as "B symptoms."
For example, a person with Hodgkin lymphoma involving lymph nodes in the neck, mediastinum, and groin (ie, involvement above and below the diaphragm) who also has symptoms of fever, night sweats, and weight loss has stage IIIB disease.
Tests used in staging — A number of tests are used in the process of staging. These tests help determine which areas of the body have been affected by Hodgkin lymphoma. However, not all patients will require every test. Tests that may be done include:
●Blood tests
●PET/CT scan of the chest, abdomen, and pelvic area
●Bone marrow biopsy – Some patients may need this procedure (a small sample of tissue is removed from the bone marrow, the spongy area in the middle of large bones) to complete disease staging.
HODGKIN LYMPHOMA TREATMENT — The main treatments for Hodgkin lymphoma are chemotherapy and radiation.
Chemotherapy — Chemotherapy is a treatment given to stop the growth of cancer cells. Most treatments involve a combination of several chemotherapy drugs (called regimens). Most of the drugs are given into the vein (intravenous, IV).
Chemotherapy is not typically given every day but instead is given in cycles. A cycle of chemotherapy (which is typically 21 or 28 days) refers to the time it takes to give the treatment and then allow the body to recover from the side effects of the medicines.
For example, two doses of ABVD chemotherapy (see below) are given 14 days apart to make up one cycle of treatment. If this regimen were repeated for a total of four cycles, it would take up to four months to complete.
Chemotherapy regimens for Hodgkin lymphoma include:
●ABVD – ABVD includes Adriamycin (doxorubicin), bleomycin, vinblastine, and dacarbazine. ABVD is the most commonly used chemotherapy regimen.
●BV+AVD – This regimen is similar to ABVD, but it substitutes a drug called brentuximab vedotin (BV) for bleomycin.
●BEACOPP – BEACOPP includes bleomycin, etoposide, Adriamycin, cyclophosphamide, Oncovin (vincristine), procarbazine, and prednisone. This regimen is more commonly used in Europe. Some experts feel that BEACOPP is more effective than other regimens, especially in people with more advanced disease. However, it has more toxic side effects, which some people cannot tolerate.
Side effects — The type and severity of chemotherapy side effects depend on the combination and dose of chemotherapy medicines given. The most common treatment-related side effects include temporary hair loss, nausea, vomiting, constipation, fatigue, loss of appetite, increased risk of infections, and neuropathy. Many of these side effects can be prevented or treated.
Long-term side effects of chemotherapy include:
●Heart problems – Adriamycin (doxorubicin) can weaken the heart muscle in some people.
●Lung damage – Bleomycin, which is used in all treatment regimens, can damage the lungs.
●Infertility – Some types of chemotherapy can damage a woman's oocytes (eggs). If most or all of the eggs are damaged or destroyed, a woman will go through an early menopause. This means that her periods will stop permanently, and she will have infertility because her eggs are damaged or gone.
Early menopause, sometimes called "premature ovarian failure" or "primary ovarian insufficiency," is most common with BEACOPP chemotherapy. About 50 percent of women lose their periods permanently after receiving BEACOPP. In contrast, ABVD chemotherapy does not seem to damage the ovaries.
The pattern is similar in men. BEACOPP chemotherapy causes very low sperm counts and infertility, but ABVD treatment does not.
●Secondary cancer – There is a risk of developing a second cancer years after the first treatment for Hodgkin lymphoma. The most common secondary cancers include those of the breast, lung, or gastrointestinal system. (See "Second malignancies after treatment of classic Hodgkin lymphoma".)
Some forms of radiation therapy increase the risk of developing cancer in areas that were treated (such as to the lungs and breast tissue) years after therapy is finished. Women under 30 are at increased risk of breast cancer, and smokers have an increased risk of lung cancer (beyond the already increased risk of lung cancer from smoking). It is also possible to develop leukemia or other types of lymphoma.
Radiation therapy — Radiation therapy (RT) involves using high-energy X-rays to stop the growth of cancer cells. Unlike normal cells, cancer cells cannot repair the damage caused by exposure to X-rays over several weeks.
Radiation therapy is sometimes recommended in stage I to II and occasionally recommended in stage III to IV to treat Hodgkin lymphoma, usually after finishing chemotherapy. In the past, radiation was directed to the area of affected lymph nodes with a carefully focused beam of radiation; this is called involved field radiation. Radiation oncologists now use involved site or involved nodal irradiation, which involve smaller treatment volumes than involved field radiation. Radiation therapy must be given in small daily doses over a period of weeks to minimize the side effects.
Side effects — During radiation treatment, some people develop skin changes in the area that was treated, similar to a sunburn. These changes fade over time. Other side effects can include a sore throat or soreness on swallowing, temporary loss of taste and thickness of saliva, fatigue, and sometimes nausea.
You and your healthcare provider should discuss the risks and benefits of radiation therapy when deciding on a treatment plan. Limiting the radiation dose and area treated can reduce, but not totally eliminate, these risks. (See "Second malignancies after treatment of classic Hodgkin lymphoma".)
Stage I or II — People with stage I or II disease are treated with chemotherapy with radiation or with chemotherapy alone. (See "Treatment of relapsed or refractory classic Hodgkin lymphoma" and "Treatment of unfavorable prognosis early (stage I-II) classic Hodgkin lymphoma in adults".)
Using chemotherapy along with radiation has a lower relapse rate than using chemotherapy alone, though it does not appear to change survival. However, having chemotherapy and radiation therapy can have more late side effects. Discuss the risks and benefits of these options with your doctor or nurse; your understanding and involvement are an important part of your cancer care.
The challenge of managing Hodgkin lymphoma is that there are risks of long-term complications related to the treatment, which can affect survival. After finishing treatment for Hodgkin lymphoma, you should work with your doctor to monitor for and prevent new cancers.
Stage III and IV — The most common treatment for people with stage III and IV Hodgkin lymphoma is chemotherapy. Radiation therapy may be added if the tumor was large at the time of diagnosis or if there are areas of tumor that have not completely responded to chemotherapy. (See "Initial treatment of advanced (stage III-IV) classic Hodgkin lymphoma".)
Most people with stage III and IV disease will get BV-AVD or ABVD. Approximately 80 percent of people will have a complete response after treatment with ABVD. Depending on your situation, medical history, and preferences, your doctor might suggest one of the other regimens (A+AVD, BEACOPP, or Stanford V with radiation) instead. (See 'Chemotherapy' above.)
Stem cell transplantation — Stem cell transplantation (also known as bone marrow transplantation or hematopoietic stem cell transplantation) might be offered to people who have:
●Recurrent Hodgkin lymphoma
●Hodgkin lymphoma that is resistant to other forms of treatment. This includes people whose disease relapses after the first round of treatment.
A separate article discusses stem cell transplantation. (See "Patient education: Hematopoietic cell transplantation (bone marrow transplantation) (Beyond the Basics)" and "Hematopoietic cell transplantation in classic Hodgkin lymphoma".)
Relapsed disease — For Hodgkin lymphoma that has returned after initial treatment, options include additional chemotherapy with a new regimen, stem cell transplantation, targeted therapy, immunotherapy, and radiation therapy. Targeted therapy involves a medication that works by affecting ("targeting") specific cancer cells. Immunotherapy is the term for drugs that work with your immune system to stop or slow the growth of cancer cells. A combination of treatments may be used.
The different approaches to treating relapsed Hodgkin lymphoma are being studied to better understand the long-term effects.
CLINICAL TRIALS — Many people with Hodgkin lymphoma will be asked about enrolling in a clinical (research) trial. A clinical trial is a carefully controlled way to study the effectiveness of new treatments or new combinations of known therapies. Ask your doctor or nurse for more information, or read about clinical trials at:
●www.cancer.gov/about-cancer/treatment/clinical-trials
Videos addressing common questions about clinical trials are available from the American Society of Clinical Oncology (www.cancer.net/research-and-advocacy/clinical-trials/welcome-pre-act).
WHERE TO GET MORE INFORMATION — Your healthcare provider is the best source of information for questions and concerns related to your medical problem.
This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.
Patient level information — UpToDate offers two types of patient education materials.
The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.
Patient education: Hodgkin lymphoma in adults (The Basics)
Patient education: Hodgkin lymphoma in children (The Basics)
Patient education: Lymphoma (The Basics)
Patient education: Neutropenia and fever in people being treated for cancer (The Basics)
Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.
Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.
Nodular lymphocyte-predominant Hodgkin lymphoma: Clinical manifestations, diagnosis, and staging
Hodgkin lymphoma: Epidemiology and risk factors
Hematopoietic cell transplantation in classic Hodgkin lymphoma
Clinical presentation and diagnosis of classic Hodgkin lymphoma in adults
Initial treatment of advanced (stage III-IV) classic Hodgkin lymphoma
Management of classic Hodgkin lymphoma during pregnancy
Monitoring of the patient with classic Hodgkin lymphoma during and after treatment
Overview of Hodgkin lymphoma in children and adolescents
Pathology of mediastinal tumors
Second malignancies after treatment of classic Hodgkin lymphoma
Pretreatment evaluation, staging, and treatment stratification of classic Hodgkin lymphoma
Pathogenesis of Hodgkin lymphoma
Treatment of favorable prognosis early (stage I-II) classic Hodgkin lymphoma
Treatment of nodular lymphocyte-predominant Hodgkin lymphoma
Treatment of relapsed or refractory classic Hodgkin lymphoma
Treatment of unfavorable prognosis early (stage I-II) classic Hodgkin lymphoma in adults
The following organizations also provide reliable health information:
●National Cancer Institute
(www.cancer.gov/types/lymphoma)
●American Society of Clinical Oncology
