Hodgkin Lymphoma
What is Hodgkin Lymphoma?
Hodgkin lymphoma is a cancer of the lymphatic system. The lymphatic system is part of the immune system.
It consists of organs and tissues, including:
- Lymph nodes
- Lymphatic vessels
- Tonsils
- Thymus
- Bone marrow
- Spleen
These tissues produce, store, and carry white blood cells called lymphocytes. They fight infection and disease.
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:
- Lymphatic fluid: Colorless, watery fluid that carries white blood cells called lymphocytes. Lymphocytes protect the body against certain types of infections.
- Lymph vessels: A network of thin tubes that collect lymphatic fluid and return it to the bloodstream.
- Lymph nodes: Small, bean-shaped structures that filter lymphatic fluid 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 in the neck, underarm, abdomen, pelvis, and groin.
- Spleen: The spleen is on the left side of the abdomen near the stomach. This organ has many jobs. It:
- Makes lymphocytes
- Filters the blood
- Stores blood cells
- Destroys old blood cells
- Thymus: An organ in which lymphocytes grow and multiply. The thymus is 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 is the type of lymphocyte involved. The lymphoma is Hodgkin lymphoma if testing on the biopsy tissue show certain tumor markers along with larger atypical cells called the Hodgkin Reed-Sternberg cell. Reed-Sternberg cells are distinctive because they have two nuclei. Some say these cells 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.
Subtypes of Hodgkin Lymphoma
Within Hodgkin lymphoma, there are two major subtypes: classical and nodular lymphocyte predominant.
- Classical Hodgkin lymphoma is the most common. There are 4 kinds of classical Hodgkin lymphoma and all are positive for the tumor marker CD30.
- Lymphocyte-rich
- Nodular sclerosis
- Mixed cellularity
- Lymphocyte-depleted
- Nodular lymphocyte predominant Hodgkin lymphoma. This subtype is positive for the tumor marker CD20 and is negative for CD30.
Knowing the specific type of Hodgkin lymphoma is very important to help doctors prescribe the best type of treatment. All kinds of classical Hodgkin lymphomas are treated the same. Nodular lymphocyte-predominant Hodgkin lymphoma has different markers. It is more slow-growing. It allows for a different treatment approach.
How common is Hodgkin lymphoma?
Some 6,000-7,000 new cases of Hodgkin lymphoma are diagnosed each year in the United States.
What are the causes/ risk factors for childhood Hodgkin lymphoma?
Causes and risk factors include:
- Age: Hodgkin lymphoma in childhood 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), the virus that causes mononucleosis, has been linked to Hodgkin lymphoma. Many people get EBV. But very few develop Hodgkin lymphoma.
- Weakened immune system: A person with a weakened immune system can be at higher risk. Immune problems may be caused by an inherited disease, certain medications, or human immunodeficiency virus (HIV) infection.
- Family history: Having a brother, sister, or parent with Hodgkin lymphoma slightly elevates the risk of developing Hodgkin lymphoma. But it does not make it harder to treat.
Signs and Symptoms of Hodgkin Lymphoma
The most common symptom 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 for no other reason
- 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
Diagnosis of Hodgkin Lymphoma
- Physical exam and medical history
- Blood tests
- X-ray and other imaging tests
- Biopsy
- Bone marrow tests
- Tests to see if the cancer has spread
A biopsy of lymph node tissue is required to make a diagnosis of Hodgkin lymphoma.
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The patient will typically receive a physical exam. 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. The provider will also take a medical history.
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Blood tests may include a complete blood count (CBC) and blood chemistry study.
Most blood work is normal in Hodgkin lymphoma. But sometimes patients have an elevated ESR (erythrocyte sedimentation rate) or CRP (C-reactive protein) level. Some may have low levels of albumin at diagnosis.
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About two-thirds of Hodgkin lymphoma patients develop a mass in the chest 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. 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 a mediastinoscope. It is a thin, tube-like instrument used to examine and remove tissue and lymph nodes in the area between the lungs. Doctors will use this method 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 indicate classical Hodgkin lymphoma.
If abnormal cells are present, the pathologist will perform more testing on the tissue sample to look for specific markers present on the atypical cancer cells. This process can take a few days.
Doctors will conduct more tests to determine the stage of the disease. The stage indicates where the cancer is located in the body.
The meaning of the stage in Hodgkin lymphoma is a little different because lymph nodes throughout the body are connected. Cancer may appear in several or many places, but it does not make it harder to treat or more risky like in other types of cancer. 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 1 or more lymph nodes in one lymph node group |
| Stage 2 | In 2 or more lymph node groups only on one side of the diaphragm. Either above OR below. |
| 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, lungs, or bone marrow. The cancer has spread to these areas from more distant places in the body. |
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 for no other reason
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 is located in the bone marrow. Sometimes imaging 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. 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 is different than in adults. The goal is to use the least amount of treatment possible for cure. 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. 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).
Cancer centers use several medicines in different combinations to treat 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 to find the most effective therapies.
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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Some patients may also have radiation therapy as part of their treatment. In the past, every patient received radiation therapy after finishing chemotherapy because it works very well to treat Hodgkin lymphoma. Today, it 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 take 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 at the end of all chemotherapy as part of their cure.
Patients whose cancer has responded well may not need radiation.
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.
Targeted therapy and immunotherapy
Targeted therapy is used in some cases of Hodgkin lymphoma. Brentuximab vedotin and rituximab are examples. Several others are under study. Brentuximab vedotin targets the CD30 marker. Rituximab targets CD20.
Targeted immunotherapy
Pembrolizumab is a targeted immunotherapy drug that is sometimes a part of treatment for relapsed or refractory Hodgkin lymphoma. It is a PD1 inhibitor. It blocks a pathway in cells called PD1. This pathway can allow cancer cells to hide from the immune system. If the pathway is blocked, it can keep cancer cells from hiding and allow the immune system to destroy cancer cells.
Hematopoietic cell transplant
Patients whose Hodgkin lymphoma doesn’t respond to treatment or returns after treatment may have a hematopoietic cell transplant (sometimes called bone marrow transplant or stem cell transplant).
Surgery
Surgery is not commonly used in Hodgkin lymphoma except to perform the initial biopsy to make the diagnosis. However it may be performed for cases of nodular lymphocyte-predominant Hodgkin lymphoma if it is possible to safely remove all cancerous lymph nodes.
Treatment usually takes 2-6 months. The length of treatment depends on the risk group of the Hodgkin lymphoma and if radiation therapy is needed, it is performed at the end of all chemotherapy and generally takes about 3 weeks.
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 have surgery to get a central venous catheter for chemotherapy, fluids, and blood samples.
- Fertility counseling
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
Doctors usually try to start treatment between 2 weeks-1 month from diagnosis. This allows time for diagnostic test results to determine the best approach. Hodgkin lymphoma is a slow-growing cancer.
Hodgkin lymphoma patients generally begin with 2 cycles of chemotherapy.
Then patients have imaging tests, usually a PET scan and a CT scan or MRI scan, to see how the cancer has responded to therapy. Treatment response determines if the patient also needs radiation treatment as part of their cure.
- Patients who have responded well to initial chemotherapy may not need radiation.
- Patients whose cancer has not responded well will 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. They may need radiation treatment. Radiation generally takes about 3 weeks.
Intermediate-risk patients
- 2 more cycles of chemotherapy with or without radiation treatment at the end.
High-risk patients
- 4 more cycles of chemotherapy with or without radiation treatment at the end.
- Treatment for high-risk cases generally takes 6-9 months with possibly some rest weeks built in during cycles to allow the body to recover depending on treatment plan.
Follow-Up Schedule
Year 1-2
The patient may return for follow-up visits once every 3-4 months for the first 2 years.
These visits may include:
- Physical examination and medical history
- Blood tests and other laboratory tests
- Imaging tests (sometimes, but less frequently needed now)
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 U.S. 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.
Long-term and late effects depend on what medicines were used, 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 rare cases when children’s cancer doesn’t respond to the original therapy or returns after treatment.
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Reviewed: September 2019