Hodgkin Lymphoma
What is Hodgkin Lymphoma?
Hodgkin lymphoma is a cancer of the lymphatic system, which is part of the immune system. The lymphatic system is made up of organs and tissues, including the lymph nodes, lymphatic vessels, tonsils, bone marrow, spleen and thymus. These tissues produce, store, or carry white blood cells called lymphocytes that fight infection and disease.
Parts of the lymphatic system:
- Lymph: Colorless, watery fluid that carries white blood cells called lymphocytes through the lymph system. Lymphocytes protect the body against certain types of infections.
- Lymph vessels: A network of thin tubes that collect lymph from different parts of the body and return it to the bloodstream.
- Lymph nodes: Small, bean-shaped structures that filter lymph and store white blood cells that help fight infection and disease. Lymph nodes are located along the network of lymph vessels found throughout the body. Clusters of lymph nodes are found in the neck, underarm, abdomen, pelvis, and groin.
- Spleen: An organ that makes lymphocytes, filters the blood, stores blood cells, and destroys old blood cells. The spleen is located on the left side of the abdomen near the stomach.
- Thymus: An organ in which lymphocytes grow and multiply. The thymus is located in the chest behind the breastbone.
- Tonsils: Two small masses of lymph tissue at the back of the throat. The tonsils make lymphocytes.
- Bone marrow: The soft, spongy tissue in the center of large bones. Bone marrow makes white blood cells, red blood cells, and platelets.
- Peyer’s patches: Small areas of lymphatic tissue located in the small intestine.
There are two types of lymphoma: Hodgkin and non-Hodgkin.
The main difference between Hodgkin and non-Hodgkin lymphoma is the type of lymphocyte involved. If tests show that an abnormal cell known as the Hodgkin Reed-Sternberg cell is present, the lymphoma is classified as Hodgkin lymphoma. This cell is distinctive because it has two nuclei, which some say have the appearance of “owl eyes.”
The lymphatic system is made up of organs and tissues, including the lymph nodes, lymphatic vessels, tonsils, bone marrow, spleen and thymus.
Reed-Sternberg cells are distinctive because they have two nuclei, which some say have the appearance of “owl eyes.” The presence of Reed-Sternberg cell classifies the lymphoma as Hodgkin lymphoma.
Within Hodgkin lymphoma, there are two major subtypes:
- Classical Hodgkin lymphoma - the most common type
- Nodular lymphocyte predominant Hodgkin lymphoma
Identifying the specific type of Hodgkin lymphoma is important because the treatment for each of these two types can be different.
There are 4 kinds of classical Hodgkin lymphoma:
- Lymphocyte-rich
- Nodular sclerosis
- Mixed cellularity
- Lymphocyte-depleted
Knowing the specific type of Hodgkin lymphoma helps doctors prescribe the best treatment for each patient. Classical Hodgkin lymphomas are treated the same. Nodular lymphocyte-predominant Hodgkin lymphoma is sometimes treated differently.
How common is Hodgkin lymphoma?
Some 6,000-7,000 new cases of Hodgkin lymphoma are diagnosed each year in the United States. About 10 to 15 percent of cases occur in children and teenagers.
What are the causes/ risk factors for childhood Hodgkin lymphoma?
Causes and risk factors include:
- Age: Childhood Hodgkin lymphoma most often affects teens ages 15-19. It also is more common in young adults and adults older than 50.
- Viral infections: Epstein-Barr virus (EBV) has been linked to Hodgkin lymphoma.
- Weakened immune system: A person with a weakened immune system can be more likely to develop Hodgkin lymphoma. Immune deficiencies may be caused by an inherited disease, medications that suppress the immune system, or human immunodeficiency virus (HIV) infection.
- Family history: Having a brother, sister, or parent who also has or has had Hodgkin lymphoma elevates the risk slightly.
The lymphatic system is a network of nodes, glands, and vessels that transports white blood cells through the body to fight infection.
Signs and Symptoms of Hodgkin Lymphoma
The most common symptom of Hodgkin lymphoma is painless, swollen lymph nodes in the neck, chest, armpit, or groin area.
Other common symptoms include:
- Weight loss of more than 10 percent of total body weight over 6 months.
- Drenching night sweats (so severe that bed sheets or pajamas may need to be changed).
- Fevers more than 100.4 degrees Fahrenheit (38 degrees Celsius) for 3 or more consecutive days that have no known cause.
- Unexplained cough or shortness of breath
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At the pediatrician’s office, the patient will typically receive a physical exam and a medical history will be taken. During the physical exam, the doctor will check general signs of health and feel for any lumps or lymph nodes that seems unusual. The doctor may feel the abdomen for signs of an enlarged spleen or liver.
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Blood tests may include a complete blood count (CBC) and blood chemistry study. A CBC looks at the number of red blood cells, white blood cells, platelets, hematocrit, and hemoglobin. During blood chemistry studies, the patient’s blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. A higher or lower than normal amount of a substance can indicate disease. Most blood work is normal in Hodgkin lymphoma, but sometimes patients may have an elevated ESR (erythrocyte sedimentation rate) or CRP (C-reactive protein) level. Some may have low levels of albumin at initial diagnosis.
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X-rays of the chest area will show the heart, lungs, lymph nodes, diaphragm, spine, ribs, collarbone, and breastbone. About two-thirds of Hodgkin lymphoma develop a mass in the chest (mediastinal) area that is visible on an X-ray.
Biopsy
A surgeon will perform a biopsy to remove tissue from an enlarged lymph node. Pathologists examine the tissue under a microscope and provide a diagnosis.
The type of biopsy depends on the location of the suspected cancer:
- Excisional biopsy: The removal of an entire lymph node or lump of tissue. This type of biopsy is preferred so the pathologist can see the architecture of the node to help make the diagnosis.
- Incisional biopsy: The removal of part of a lump, lymph node, or tissue sample.
- Core biopsy: The removal of small pieces of tissue or part of a lymph node using a wide needle.
- Fine-needle aspiration biopsy: The removal of tissue or part of a lymph node using a thin needle.
If Hodgkin lymphoma involves lymph nodes deep in the chest, a biopsy may involve use of a mediastinoscope, a thin, tube-like instrument used to examine and remove tissue and lymph nodes in the area between the lungs if there are no other lymph nodes that are easier to sample.
Analysis of biopsy tissue
The pathologist will examine the tissue to look for cancer cells. Hodgkin Reed-Sternberg cells are characteristic of classical Hodgkin lymphoma. If abnormal cells are present, the pathologist will perform additional testing (immunohistochemistry) on the tissue in order to look for specific markers present on the cancer cells under the microscope. After a biopsy confirms cancer, doctors will conduct more tests to determine the stage of the disease. The stage indicates whether the cancer has spread to other parts of the body and, if so, how much and how far it has spread.
Staging in Hodgkin lymphoma is a little different because lymph nodes throughout the body are connected so the cancer often will appear in a number of places. Staging in Hodgkin lymphoma depends on:
- The number of affected lymph node regions
- The patient’s signs and symptoms
- If the tumor is bulky
- If lymphoma has spread outside the lymphatic system
| Stage | Where Cancer is Found |
|---|---|
| Stage 1 | In one or more lymph nodes in one lymph node group |
| Stage 2 | In two or more lymph node groups only on one side of the diaphragm (either above OR below the thin muscle below the lungs that helps |
| Stage 3 | In lymph node groups above AND below the diaphragm |
| Stage 4 | In areas of the body that are not part of the lymph node system, such as the liver or lungs. In the lung, liver, bone marrow. |
A or B designations are also used in addition to the stage for every patient.
A means you have no “B” symptoms.
B is added if a patient has the presence of at least one of the following symptoms:
- Drenching night sweats
- Unexplained fevers with temperature more than 38°C or 100.4°F for 3 consecutive days
- More than 10% loss of total body weight during the past 6 months
E is used when the cancer is found outside the lymph system in one organ or area.
Imaging tests for staging at the time of initial diagnosis may include:
- Chest X-ray
- PET scan
- CT scan or MRI scan
- Ultrasound
Bone marrow aspiration and biopsy
A bone marrow aspiration and biopsy may be performed to see if cancer has spread to parts of the bone marrow. Sometimes imaging tests alone can provide this information and the procedure is not needed.
Risk groups
Risk groups are determined at diagnosis and are used to plan treatment. Doctors determine risk groups based on the signs, symptoms, and stage of the cancer. For Hodgkin lymphoma, all risk groups have the same outcomes if they receive the appropriate treatment based on risk grouping. This is why it is so important to accurately identify the risk group through correct staging.
Risk groups:
- Low
- Intermediate
- High
Treatment of Hodgkin Lymphoma
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The main treatment for Hodgkin lymphoma is chemotherapy. The treatment approach in children differs from that in adults. The goal is to use the least amount of treatment possible to cure Hodgkin disease. Using less treatment helps to prevent long-term and late effects of treatment.
The most common treatment plan for Hodgkin lymphoma in adults is called ABVD. It is not commonly used in children because many cycles of this combination are highly likely to cause serious long-term heart damage later in life. ABVD is a combination of four drugs -- Adriamycin® (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine (DTIC).
Today, cancer centers use several medicines in different combinations to treat Hodgkin lymphoma in children. Different drugs successfully treat cancer by fighting it in different ways. This approach uses less of each drug, which can help prevent side effects, including long-term and late effects.
In the U.S., common chemotherapy combinations for pediatric Hodgkin disease include:
- BEACOPP: Bleomycin, etoposide, doxorubicin (Adriamycin®), cyclophosphamide, vincristine (Oncovin), procarbazine, and prednisone
- Stanford V: Doxorubicin, vinblastine, mechlorethamine, vincristine, bleomycin, etoposide, and prednisone.
In Europe common chemotherapy combinations include:
- OEPA: Vincristine (Oncovin), etoposide, prednisone, and doxorubicin
- COPDAC: Cyclophosphamide, vincristine, prednisone, and dacarbazine
Cancer centers may add new drugs or take away drugs in an ongoing effort to find the most effective therapies to cure the cancer while preventing side effects.
Different collaborative pediatric oncology groups have developed treatment protocols for pediatric Hodgkin lymphoma. The groups share information to improve treatment methods. Their treatment protocols all have high success rates. These groups include the Children’s Oncology Group (COG), the St. Jude-Stanford-Dana Farber Consortium, and the European Network for Pediatric Hodgkin Lymphoma Group (EuroNet-PHL).
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Radiation therapy may also be needed to cure a patient. In the past it was the standard of care for every patient to receive radiation therapy after completion of chemotherapy. Today, whether a patient receives radiation therapy depends on how the cancer responds during treatment.
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Doctors choose treatment plans based on the risk group. In general:
- Low-risk: 2 cycles of chemotherapy
- Intermediate-risk: 4 cycles of chemotherapy
- High-risk: 6 cycles of chemotherapy
- All Hodgkin lymphoma patients generally begin with 2 cycles of chemotherapy that takes about 8 weeks
- Then patients undergo imaging tests, usually a PET scan, to see how well the cancer has responded to treatment. Treatment response determines if the patient also needs radiation treatment.
Patients whose cancer has responded well may not need radiation as part of their treatment plan.
Patients whose cancer has not responded as well typically receive radiation after they finish their chemotherapy.
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In some cases, patients may receive other, additional treatments. These include:
Targeted therapy and immunotherapy
Targeted therapy is used in some cases of Hodgkin lymphoma. Brentuximab vedotin and pembrolizumab are examples. Several others are under study.
Hematopoietic cell transplant
Patients whose Hodgkin lymphoma doesn’t respond to treatment or returns after treatment may undergo a hematopoietic cell transplant (sometimes called bone marrow transplant or stem cell transplant).
Surgery
Surgery is not commonly used in Hodgkin lymphoma, except in cases of nodular lymphocyte-predominant Hodgkin lymphoma when it may be possible to remove cancerous lymph nodes.
Treatment usually takes 2-6 months. The length of treatment depends on the risk group of the Hodgkin lymphoma.
Treatment Timeline
Week 1
Workup, Treatment Prep
- Blood tests
- Physical exam and history
- Biopsy of enlarged lymph nodes
- Imaging tests to determine risk group. The risk group determines the length of therapy.
- Imaging tests may include:
- Bone marrow aspirate and/or biopsy may be needed.
- Many Hodgkin patients undergo surgery to get a PICC line to receive chemotherapy, fluids, and to take blood samples.
A PET (positron emission tomography) scan is a test that shows how organs and tissues function inside the body.
When the PET machine is turned on, the patient will see the red laser lights of the scan but won’t feel them.
Next 8 weeks
Hodgkin lymphoma patients generally begin with 2 cycles of chemotherapy.
Then patients undergo imaging tests, usually a PET scan, to see how the cancer has responded to therapy. Treatment response determines if the patient also needs radiation treatment.
- Patients who have responded well to initial chemotherapy may not need radiation therapy.
- Patients whose cancer has not responded well typically receive radiation after they finish chemotherapy.
Chemotherapy, or “chemo,” is the treatment of cancer using powerful medicines.
Radiation therapy uses beams of radiation, either X-rays or protons, to shrink tumors and kill cancer cells.
Next 2-4 months
Low-risk patients generally do not receive additional chemotherapy but may need radiation treatment. Radiation treatment generally takes about 3 weeks.
Intermediate-risk patients
- 2 more cycles of chemotherapy with or without radiation treatment
High-risk patients
- 4 more cycles of chemotherapy with or without radiation treatment.
- Treatment for high-risk cases generally takes a total of 6-9 months with possibly some short breaks.
Follow-Up Schedule
Year 1-2
The patient may return for follow-up visits once every 4 months for the first 2 years.
These visits may include:
- Physical examination and medical history
- Blood tests and other laboratory tests
Years 3-4
Follow-up visits may change to once every 6 months.
Year 5
Follow-up visits may change to once a year.
Prognosis of Hodgkin Lymphoma
The survival rate for Hodgkin lymphoma in the United States is more than 95 percent.
Late Effects of Hodgkin Lymphoma Treatment
Cancer treatments may have long-term and late effects.
Long-term effects start during treatment and continue after treatment ends.
Late effects don’t start until later in life.
What conditions occur depend on several factors, including specific medicines used during treatment, amount and location of radiation treatment, and age of the patient.
Focus of Current Hodgkin Lymphoma Research
Research continues to develop treatments that reduce the long-term and late effects in cancer survivors.
Research also focuses on developing more effective treatments for children whose cancer doesn’t respond to the original therapy or returns after treatment.
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Reviewed: June 2018