Senior Physician and Assistant Professor of Medicine at Dana-Farber Cancer Institute
Would you briefly describe Hodgkin lymphoma (HL)?
Hodgkin lymphoma (HL) is a cancer that involves the lymphoid cells (cells that live in the lymph glands). The cell HL arises out of what is called a B-cell lymphocyte, which is one of the specific types of immune cells.
HL, arises from a damaged B-cell lymphocyte, known as a Reed Sternberg, which starts to copy itself leading to a collection of abnormal cells, typically within a lymph gland. In order to make a diagnosis, doctors will perform a biopsy and will remove a piece of the lymph node or other involved tissue. A pathologist (a doctor who specializes in the diagnosis of diseases by studying the cells from a patient's tissue samples) then looks at the cells under the microscope to help determine the patient's diagnosis by identifying the appearance and pattern of the cells often in combination with evaluaiting specific proteins on and in the tumor cells. Hodgkin lymphomas have a particular look when viewed under the microscope. The Reed Sternberg cells are rare and are surrounded by normal immune cells, Most often HL presents itself in lymph nodes, but it can also appear in other sites including the bone, lung, or liver.
How common is Hodgkin lymphoma?
Hodgkin lymphoma is a relatively rare disease and there are approximately 9,000 new cases diagnosed each year in the United States. Although both children and adults can develop HL, the disease is most common in adults aged 20 to 34.
How is Hodgkin lymphoma typically treated?
Treating HL will depend on several factors such as a patient's overall health and age, preferences and goals for treatment, as well as the patient's stage of disease. Staging is used to describe how widely the lymphoma has spread in patients with HL and other types of cancer. If the lymphoma is localized in the neck or chest or in the abdomen or in the pelvis, we refer to that as early stage disease. For patients with early stage disease, the standard treatment was chemotherapy followed by radiation but now we try to avoid using radiation in patients who respond well to chemotherapy alone to prevent the long-term side effects that may result.
When lymphoma is found in other locations, it is referred to as advanced-stage disease. Patients with advanced-stage disease are always given chemotherapy. We don't typically add radiation to the treatment regimen except in some situations where the lymph node areas involved are very large, or certain areas don't respond completely to chemotherapy.
In treating patients with HL, the most commonly used chemotherapy regimen is ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) which is typically given to the patient every two weeks, for as few as two months to up to six months depending on the stage of the patient and whether or not radiation will be given upon the completion of chemotherapy.
There is another regimen that is sometimes used called escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone), which is a very intensive regimen used in Europe for advanced-stage disease. It may be used in certain circumstances in the United States, but it is less common.
Could you briefly describe stem cell transplantation?
There are two types of stem cell transplantation that can be performed on patients: autologous stem cell transplant (the patient is his or her own donor) or allogeneic stem cell transplant (the donor is another person who is genetically similar to the patient).
Autologous tem cell transplantation allows the patient to receive a much higher dose of chemotherapy with the goal of getting rid of any lymphoma cells that may have survived the standard dose of chemotherapy. In order to facilitate giving much higher doses of chemotherapy, we collect bone marrow stem cells and store the cells in the freezer. After the high dose chemotherapy has been completed , the stem cells are then given back to the patient. By doing this we protect the patient's stem cells from the effects of the chemotherapy and are able to give much higher doses of chemotherapy.
In an allogeneic stem cell transplant, the patient's bone marrow is replaced with the bone marrow of someone else that ultimately gives the patient a new immune system, which we hope will attack the lymphoma. Before performing an allogeneic stem cell transplant, a donor must first be identified. The donor can be a sibling or an unrelated adult. If a brother or a sister is not an appropriate match to the patient, we then look at the registry of people who have signed up to donate their stem cells. In some cases, when a fully matched donor cannot be identified, we can find a donor who is half a match (i.e., parent, child, sibling) and perform a haploidentical transplant.
Once a donor is identified, he or she receives white blood cell boosters for a few days and their stem cells are removed using a machine, much like donating red blood cells or platelets. The patient is then given chemotherapy drugs to suppress their immune system and then the patient receives the donor's stem cells. The new stem cells then establish themselves in the patient's bone marrow and a new immune system emerges. These stem cells know that they are in a new home and may recognize that a recipient's organs or other parts of the body as foreign and cause graph-versus-host disease (GVHD), which is a condition where the donated bone marrow attacks the patient's tissues.
Is one type of stem cell transplantation more common than the other in treating Hodgkin lymphoma?
Yes. When possible in patients with HL, we almost always try to perform an autologous stem cell transplant; it's quite rare that we perform an allogeneic stem cell transplant.
Most patients who have an allogeneic stem cell transplant have already undergone an autologous stem cell transplant. It's fairly uncommon to have recurrence of lymphoma after an autologous stem cell transplant. Recent data suggests that brentuximab vedotin (Adcetris) when given after autologous stem cell transplant may keep more patients in remission longer.
Why is stem cell transplantation important in treating patients with Hodgkin lymphoma?
The goal over time is to cure more patients with HL with initial treatment. We have seen success with the initial treatments of ABVD chemotherapy, which is a very effective combination.
The percentage of patients whose disease returns after initial treatment (known as relapsed disease) or does not respond to treatment (known as refractory disease) with ABVD is small; with new drugs such as brentuximab vedotin (Adcetris) and most recently, the PD-1 inhibitors such as nivolumab (Opdivo) and pembrolizumab (Keytruda), our hope is by initially treating patients with our best therapies, we will avoid having to perform a stem cell transplant. Stem cell transplant is important for patients whose disease has returned because it does offer the possibility of long-term remissions.
What advice would you give to a newly diagnosed patient with Hodgkin lymphoma?
I think it's important to empower patients to feel some sense of control over the situation. My advice for a newly diagnosed patient with HL is to get as much information as they can about the disease but to do so by going to reliable sources to obtain this information. Upon receiving a lymphoma diagnosis, it's completely natural for patients to go online to learn about their disease, or talk to people and get ideas about what they should be doing. I advise against reading personal blogs because there is no oversight as to what people write. They talk about their own experiences which may be very different from a patient who has been newly diagnosed. I advise my patients to look at well-written websites such as the Lymphoma Research Foundation (LRF) and also to download LRF's free mobile app called Focus On Lymphoma, which is a great tool for patients and caregivers to monitor and keep track of their treatment, medications, and test results. LRF's adolescent and young adult (AYA) lymphoma initiative, Erase Lymphoma, is also a great resource and aims to assist young lymphoma patients in addressing the unique medical challenges, psychosocial needs, and access issues they may encounter by providing expert materials and programs.
How are you involved with the Lymphoma Research Foundation (LRF), and why would you recommend that patients become involved with LRF?
I've been working with the Lymphoma Research Foundation (LRF) for many years and feel that it is a great organization that provides support and resources for people with lymphoma. I'm extremely impressed by the Foundation's educational programs and workshops because they are incredibly informative for both patients and caregivers. The Lymphoma Research Foundation hosts "Ask the Doctor" programs in various cities, and it's very empowering for patients to attend these conferences where everyone in the room has a similar story and where they can learn about their disease and also speak to people who are going through what they are going through.
The Lymphoma Research Foundation has wonderful online patient resources such as booklets, factsheets, and other educational materials. I always encourage my patients to visit LRF's website at lymphoma.org to learn more about their disease and the other wonderful resources that the Lymphoma Research Foundation offers.
The Lymphoma Research Foundation also provides support through their Scientific Advisory Board (SAB) and their philanthropic support which is very important to continue research efforts in the area of lymphoma. LRF also helps by training new junior faculty members who are interested in lymphoma and educates them on how to perform clinical research in lymphoma. Additionaly, LRF coordinates regional professional educational meetings called Lymphoma Rounds, where lymphoma specialists from different institutions get together to collaborate and discuss challenging cases. Participating in these programs is always extremely educational, provides valuable insights and it's great to see our colleagues and hear how they approach things in the area of lymphoma. This helps to ensure the best possible treatment and care for patients.