Hodgkin lymphoma, also known as Hodgkin’s disease, is a blood cancer that develops in the lymphatic system. It usually starts in the lymphocytes, which are a type of white blood cell. Because lymphocytes are found throughout the body, it can grow in other parts of the body as it travels through the blood and lymphatic system. The first and most common symptom of Hodgkin lymphoma is a painless, swollen lymph node in the neck, armpit or groin.
According to the American Cancer Society, less than 8,500 people in the United States are diagnosed with Hodgkin lymphoma each year, making it a rare type of cancer. It is most often diagnosed in young adults between the ages of 15-30, followed by individuals above 55 years of age. It is also slightly more common in men than in women.
Survival rates for Hodgkin lymphoma are high, and it is considered a very treatable cancer. There are many treatment options available today including chemotherapy, radiation and immunotherapy. If you are diagnosed with Hodgkin lymphoma, it is best to consult an oncologist who specializes in the disease.
- What Is Hodgkin Lymphoma?
- Types of HL
- HL Symptoms
- HL Causes
- HL Risk Factors
- HL Diagnosis
- HL Stages
- HL Treatment
- HL Survival
Hodgkin lymphoma is a type of cancer that affects your lymphatic system, a critical part of the body’s immune system. Also known as Hodgkin’s Disease, it is one of two main types of lymphoma, the other being non-Hodgkin lymphoma.
Both diseases begin in your lymphatic system (specifically in a type of white blood cell known as a lymphocyte). The primary difference between the two is the presence of Reed-Sternberg cells, a type of cancer cell derived from B lymphocytes. If these cells are present, the cancer is classified as Hodgkin lymphoma; if they are not present, it is diagnosed as non-Hodgkin lymphoma. Despite having similar names, these two diseases behave and are treated very differently.
There are two main types of Hodgkin lymphoma. Your treatment plan will depend on which of them you have.
Classical Hodgkin Lymphoma
This is the more common form of the disease. It features the iconic presence of Reed-Sternberg cells within the lymph nodes, and has four subtypes:
- Lymphocyte-depleted Hodgkin lymphoma
- Lymphocyte-rich Hodgkin lymphoma
- Mixed cellularity Hodgkin lymphoma
- Nodular sclerosis Hodgkin lymphoma
Nodular Lymphocyte-Predominant Hodgkin Lymphoma
This is a significantly more rare version of Hodgkin lymphoma and involves a different form of large and abnormal cells, often called popcorn cells due to their appearance.
The first and most common symptom of Hodgkin lymphoma is a painless, swollen lymph node. These can occur anywhere in the body but are usually noticed in the neck, armpit or groin. Changes to your lymph nodes will be given special attention from your doctor when looking for Hodgkin lymphoma.
Other symptoms of Hodgkin lymphoma often include one or more of the following:
- unexplained weight loss
- extreme night sweating
- recurring fevers
- constant fatigue
While the exact cause of Hodgkin lymphoma is not yet fully understood, we do know that the disease begins with mutated lymphocytes which grow and divide rapidly, pushing healthy cells out.
Many scientists believe that the Epstein-Barr virus (which is known to cause Mononucleosis) can alter lymphocyte DNA, and eventually lead to Hodgkin lymphoma. According to the American Cancer Society, elements of the Epstein-Barr Virus are found in about 25% of Reed-Sternberg cells.
Additionally, Reed-Sternberg cells release cytokines, which attract other cells into the affected lymph node and cause swelling. These additional cells then release substances that help the Reed-Sternberg cells grow even more.
There are several known risk factors for developing Hodgkin lymphoma.
Epstein-Barr Virus Infection
People who have been infected with illnesses caused by the Epstein-Barr Virus, like Mono, are at an increased risk for developing Hodgkin lymphoma. Despite this connection, the chances of contracting Hodgkin lymphoma if you have been infected with the Epstein-Barr Virus are still very low — about 1 in 1,000.
Hodgkin lymphoma is slightly more prevalent among males.
While the reasons for this are still unclear, if you have a blood relative with Hodgkin lymphoma (or any type of lymphoma), your chances of developing the disease are higher.
If your doctor suspects you might have Hodgkin lymphoma, you will likely go through a series of tests to confirm or rule out a diagnosis.
Here’s what you’re likely to encounter:
First, your doctor is likely to perform a physical exam, paying special attention to your lymph nodes. They will also likely ask about your symptoms and family medical history.
While a blood test alone cannot determine whether or not you have Hodgkin lymphoma, a complete blood count (CBC) can help doctors get a clearer picture of what’s going on and can help with determining the stage of the disease. A CBC measures the level of red blood cells, white blood cells and platelets in your blood.
Lymph Node Biopsy
If your doctor finds any swollen lymph nodes during the physical exam, they will likely order a biopsy to examine a lymph node or part of a lymph node. This is the most accurate way to test for Hodgkin lymphoma. A lymph node biopsy can include removal of the entire lymph node (excisional biopsy), or just a section of a lymph node (incisional biopsy). Depending on the size of the lymph node and its location, incisional biopsies can often be done with a local anesthetic. Excisional biopsies may require general sedation or anesthesia.
I’ve Been Diagnosed with Hodgkin Lymphoma, Now What?
Your healthcare provider will help you determine the best immediate course of action. If possible, consult an oncologist who specializes in treating Hodgkin lymphoma. With telemedicine, you may even be able to speak to a lymphoma specialist from the comfort of your own home.
It’s important to learn which subtype of the disease you have and the stage that it is in, because that will inform your treatment options. Some patients find that joining a cancer support group helps them process their diagnosis and learn from others who are on a similar journey.
Hodgkin lymphoma is staged based on the location of the cancer and how many lymph node regions are affected. The staging system used for Hodgkin lymphoma is the Lugano classification, which has four stages: I, II, III and IV.
Stages I and II refer to limited-stage Hodgkin lymphoma, meaning the disease is mostly contained to the lymph nodes.
Additionally, there are other descriptors that can be applied to Hodgkin lymphoma staging, including:
- Bulky disease: this is used to describe tumors in your chest that are ⅓ the width of your chest or more.
- A and B: when B is added to a stage (IIB, for example), it means that you are displaying B symptoms of the disease, which include rapid weight loss, unexplained fever and intense night sweats.
To learn more about Hodgkin lymphoma staging and grading, speak with your doctor. They can answer your questions and help you better understand your stage and prognosis.
There are a range of treatment options for Hodgkin lymphoma, but most patients will start with chemotherapy.
Chemotherapy is the most common treatment for patients with Hodgkin lymphoma. It consists of strong drugs that kill or damage cancer cells, and many patients will take a combination of chemotherapy medications to treat this type of lymphoma. Combinations include:
- ABVD regimen: This is the most common regimen in the United States for Hodgkin lymphoma patients. It includes adriamycin (doxorubicin), bleomycin, vinblastine and dacarbazine (DTIC).
- AAVD regimen: This regimen substitutes the immunotherapy brentuximab vedotin (Adcetris) for the bleomycin used in ABVD. AAVD has shown increased efficacy compared to ABVD but also some increased risk for infections and other side effects, so the preferred regimen should be decided individually for each patient.
- BEACOPP regimen: This option includes bleomycin, etoposide (VP-16), adriamycin (doxorubicin), cyclophosphamide, oncovin (vincristine), procarbazine and prednisone.
- Stanford V regimen: In the case of this regimen, radiation therapy is usually also given. This regimen includes doxorubicin (Adriamycin), mechlorethamine (nitrogen mustard), vincristine, vinblastine, bleomycin, etoposide and prednisone.
Radiation therapy is often used in combination with a stem cell transplant to allow you to receive higher doses of chemotherapy. The radiation therapy will damage the cancer cells and stop them from spreading, while the stem cell transplant will help your body make new, healthy bone marrow.
Immunotherapy helps a patient’s own immune system kill cancer cells using monoclonal antibodies, which are a man-made version of your body’s own antibodies, to attack a substance on the surface of lymphocytes. Immunotherapy drugs for Hodgkin lymphoma include brentuximab vedotin (Adcetris) and rituximab (Rituxan). Additionally, a type of drug known as immune checkpoint inhibitors (which allow immune cells to target cancerous cells) can be used. These include drugs such as nivolumab (Opdivo) and pembrolizumab (Keytruda).
Here are a few questions to ask when discussing treatment options with your doctor:
- What type of Hodgkin lymphoma do I have?
- What is the stage of my Hodgkin lymphoma?
- Do I need to start treatment right away?
- Which treatment option(s) do you recommend, and why?
- How long will treatment last?
- What are the risks or side effects?
- How will we know the treatment is working?
According to Surveillance, Epidemiology, and End Results (SEER) data published by the National Cancer Institute, the relative five-year survival rate from 2010 to 2016 was 87.4%, meaning that approximately 87 of 100 people with Hodgkin lymphoma were still alive five years after their diagnosis.
When the disease is localized, the five-year survival rate is 92%; if the disease has spread and is classified as regional, the 5-year survival rate is 94%; if the disease has spread distantly throughout your body the 5-year survival rate is 78%.