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Hodgkin Lymphoma

(Hodgkin's Lymphoma; Hodgkin's Disease)

By

Peter Martin

, MD, Weill Cornell Medicine;


John P. Leonard

, MD, Weill Cornell Medicine

Last full review/revision Jul 2020| Content last modified Jul 2020
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Hodgkin lymphoma is a type of lymphoma distinguished by the presence of a particular kind of cancer cell called a Reed-Sternberg cell.

  • The cause is unknown.

  • Lymph nodes enlarge but are usually not painful.

  • Other symptoms such as fever, itching, and shortness of breath can develop depending on where the cancer cells are growing.

  • A lymph node biopsy is needed for diagnosis.

  • Chemotherapy, combinations of chemotherapy and immunotherapy, and radiation therapy are used for treatment.

  • Most people are cured.

Lymphomas are cancers of a specific type of white blood cells known as lymphocytes. These cells help fight infections. Lymphomas can develop from either B or T lymphocytes. T lymphocytes are important in regulating the immune system and in fighting viral infections. B lymphocytes produce antibodies, which are essential in fighting off some infections.

In the United States, about 8,000 new cases of Hodgkin lymphoma occur every year. The disease is more common in males than in females—about three men are affected for every two women. Hodgkin lymphoma rarely occurs before age 10. It is most common in people between the ages of 15 and 40 and in people older than 60.

Causes

The cause of Hodgkin lymphoma is unknown, but exposure to the Epstein-Barr virus may play a role in some people. There may also be a hereditary association. Although there are some families in which more than one person has Hodgkin lymphoma, it is not contagious. There are no genetic screening tests, and routine screening of children or siblings is not recommended.

Other people at risk for Hodgkin lymphoma include those with

Symptoms

People with Hodgkin lymphoma usually become aware of one or more enlarged lymph nodes, most often in the neck but sometimes in the armpit or groin. Although usually painless, in rare cases the enlarged lymph nodes may be painful for a few hours after a person drinks alcoholic beverages.

People with Hodgkin lymphoma sometimes experience fever, night sweats, and weight loss. They can also have itching and fatigue. Some people have an unusual pattern of high temperature for several days alternating with normal or below-normal temperature for days or weeks. This is sometimes referred to as Pel-Ebstein fever. Other symptoms may develop, depending on where the cancerous cells are growing. For example, enlargement of lymph nodes in the chest may partially narrow and irritate airways, resulting in a cough, chest discomfort, or shortness of breath. Enlargement of the spleen or lymph nodes in the abdomen may cause discomfort in the abdomen.

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Symptoms of Hodgkin Lymphoma

Symptoms*

Cause

Weakness and fatigue resulting from too few red blood cells (anemia)

Infection and fever, resulting from too few white blood cells

Bleeding, resulting from too few platelets

Possibly bone pain when the bone is involved

Lymphoma cells are invading the bone marrow.

Loss of muscle strength

Hoarseness

Enlarged lymph nodes are compressing nerves in the spinal cord or nerves to the vocal cords.

Lymphoma cells are blocking the flow of bile from the liver.

Swelling of legs and feet (lymphedema)

Lymphoma cells are blocking the flow of lymph fluid from the legs.

Cough and shortness of breath

Lymphoma cells are invading the lungs.

Decreased ability to fight infection and increased susceptibility to fungal and viral infections

Lymphoma causes dysfunction of the immune system.

* Some of these symptoms may occur for more than one reason.

Diagnosis

  • Lymph node biopsy

Doctors suspect Hodgkin lymphoma when a person with no apparent infection develops persistent and painless enlargement of lymph nodes that lasts for several weeks. The suspicion is stronger when lymph node enlargement is accompanied by fever, night sweats, and weight loss. Rapid and painful enlargement of lymph nodes—which may occur when a person has a cold or infection—is not typical of Hodgkin lymphoma. Sometimes enlarged lymph nodes deep within the chest or abdomen are found unexpectedly when a chest x-ray or computed tomography (CT) scan is done for another reason.

Abnormalities in blood cell counts and other blood tests may provide supportive evidence. However, to make the diagnosis, doctors must do a biopsy of an affected lymph node to see if it is abnormal and if Reed-Sternberg cells are present. Reed-Sternberg cells are large cancerous cells that have more than one nucleus (a structure inside a cell that holds the cell's genetic material). Their distinctive appearance can be seen when a biopsy specimen of lymph node tissue is examined under a microscope.

The type of biopsy depends on which node is enlarged and how much tissue is needed. Doctors must remove enough tissue to be able to distinguish Hodgkin lymphoma from other disorders that can cause lymph node enlargement, including non-Hodgkin lymphomas, infections, inflammation, or other types of cancer.

The best way to obtain enough tissue is with an excisional biopsy (a small incision made to remove a piece of the lymph node). Occasionally, when an enlarged lymph node is close to the body's surface, a sufficient amount of tissue can be obtained by inserting a hollow needle (under ultrasound or CT guidance) through the skin and into the lymph node (core needle biopsy). When an enlarged lymph node is deep inside the abdomen or chest, surgery may be needed to obtain a piece of tissue.

Staging

  • Imaging studies

  • Sometimes, bone marrow biopsy

Before treatment is started, doctors must determine how extensively the lymphoma has spread—the stage of the disease. The choice of treatment and the expected outcome of treatment (prognosis) depend on a variety of factors, including the stage. An initial examination may detect only a single enlarged lymph node, but procedures to find if and where the lymphoma has spread (staging) may detect considerably more disease.

The disease is classified into four stages based on the extent of its spread (I, II, III, IV). The higher the number, the more the lymphoma has spread. Limited stage disease includes stages I and II; advanced stage disease includes stages III and IV. In stages I and II, if Hodgkin lymphoma is present in an organ outside the lymph system, it is classified as stage IE or IIE. Bulky disease is the term used when there is a mass in the chest greater than 10 cm (about 4 inches) in diameter.

The four stages are subdivided, based on the absence (A) or presence (B) of one or more of the following symptoms:

  • Unexplained fever (more than 100° F [about 37.5° C] for 3 consecutive days)

  • Night sweats

  • Unexplained loss of more than 10% of body weight in the preceding 6 months

For example, a person with stage II lymphoma who has experienced night sweats is said to have stage IIB Hodgkin lymphoma.

Several procedures are used to stage or assess Hodgkin lymphoma. Basic blood tests, including liver and kidney function tests, tests for human immunodeficiency virus (HIV) and hepatitis B and hepatitis C infection, and imaging with computed tomography (CT) combined with positron emission tomography (PET) of the neck, chest, abdomen, and pelvis are standard.

Combined PET-CT is the most sensitive technique for determining the stage of Hodgkin lymphoma and for evaluating the person's response to treatment. Because living tissue can be identified with PET, doctors can use this imaging technique to distinguish scar tissue from active Hodgkin lymphoma after the person has undergone treatment (although PET is not always accurate because inflammation can also be detected by PET).

If combined PET-CT scan is not available, a contrast CT scan of the neck, chest, abdomen, and pelvis and a bone marrow biopsy are done instead. Other tests such as magnetic resonance imaging (MRI) of the brain or spinal cord are done if symptoms of the nervous system are present.

Most people with Hodgkin lymphoma do not need surgery to determine whether the disorder has spread to the abdomen because of the accuracy of the PET scan and the fact that all people receive chemotherapy, which treats the lymphoma no matter where it is located.

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Stages of Hodgkin Lymphoma

Stage

Extent of Spread

Likelihood of Cure*

I

Limited to one lymphatic tissue site (lymph node, thymus, or spleen), or only one site outside the lymphatic system (IE)†

More than 90%

II

Involves two or more lymph node regions on the same side of the diaphragm, above or below it (for example, some enlarged nodes in the neck and some in the armpit), or extension outside an involved lymph node area with or without other lymph node areas on the same side of the diaphragm (IIE)†

More than 80%

III

Involves spleen and lymph node regions above and below the diaphragm (for example, some enlarged nodes in the neck and some in the groin)

70 to 80%

IV

Involves other parts of the body (such as the bone marrow, lungs, or liver), as well as lymph nodes

More than 60%

* People have survived for 5 years with no further disease.

† In stages I and II, if Hodgkin disease is present in an organ outside the lymph system, it is classified as Stage IE or IIE.

Treatment

  • Chemotherapy

  • Radiation therapy

  • Surgery

  • Sometimes stem cell transplantation

With chemotherapy, with or without radiation therapy, most people who have Hodgkin lymphoma can be cured.

Pretreatment strategies

Before treatment and where applicable, men should be offered sperm banking, and women should discuss options to preserve fertility with their oncologists and a fertility specialist.

Chemotherapy with or without radiation therapy

Chemotherapy is used for all stages of disease. Doctors usually use more than one chemotherapy drug. Several combinations may be used and may include immunotherapy drugs as well. Immunotherapy drugs are made up of antibodies that attack cancer cells. Involved field radiation therapy (radiation therapy delivered only to the affected areas of the body, avoiding exposing unaffected areas to radiation) may be added after chemotherapy. Radiation therapy is usually given on an outpatient basis over about 3 to 4 weeks.

About 85% of people with stage I or stage II disease are cured with chemotherapy alone or with chemotherapy plus involved field radiation therapy. The cure rate of people with stage III disease ranges from 70 to 80%. Cure rates for people with stage IV disease, while not as high, are above 60%.

Although chemotherapy greatly improves the chances for a cure, side effects can be serious. The drugs may cause

  • Temporary or permanent infertility

  • An increased risk of infection

  • Potential damage to other organs, such as the heart or lungs

  • Reversible hair loss

Posttreatment strategies

After radiation therapy, there is an increased risk of cancer, such as lung, breast, or stomach cancer, occurring 10 or more years after treatment in organs that were in the radiation field. Non-Hodgkin lymphomas and leukemias may develop in some people many years after successful treatment for Hodgkin lymphoma, regardless of the treatment used.

A person who has a remission (the disease under control) after initial treatment but then relapses (lymphoma cells reappear) may still be cured with second-line treatment. The cure rate for people who relapse is at least 50%. Among people who relapse in the first 12 months after initial treatment, cure rates are somewhat lower, whereas the rates for people who relapse later tend to be somewhat higher.

People who relapse after initial treatment generally are treated with a “salvage” chemotherapy regimen followed by high-dose chemotherapy and autologous stem cell transplantation, which involves using the person's own stem cells. High-dose chemotherapy with stem cell transplantation is generally a safe procedure, with less than a 1 to 2% risk of death related to the treatment. Immunotherapy drugs are also used to treat people who have a relapse. e

After treatment has been finished, people have regular doctor's examinations and tests to look for return of the lymphoma (posttreatment surveillance) for a period of 5 years. Tests typically include blood tests and CT scans of the chest and pelvis. If people have had radiation therapy, doctors also do tests, such as mammography or magnetic resonance imaging (MRI) of the breasts and thyroid tests, to see if a new cancer developed in those organs.

More Information

The following is an English-language resource that may be useful. Please note THE MANUAL is not responsible for the content of this resource.

NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version
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