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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:
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.”
Within Hodgkin lymphoma, there are two major subtypes:
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:
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.
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.
Causes and risk factors include:
The most common symptom of Hodgkin lymphoma is painless, swollen lymph nodes in the neck, chest, armpit, or groin area.
Other common symptoms include:
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.
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.
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.
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:
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.
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:
|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:
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:
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 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.
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:
In Europe common chemotherapy combinations include:
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).
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.
Doctors choose treatment plans based on the risk group. In general:
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.
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 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.
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.
Low-risk patients generally do not receive additional chemotherapy but may need radiation treatment. Radiation treatment generally takes about 3 weeks.
The survival rate for Hodgkin lymphoma in the United States is more than 95 percent.
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.
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