Sam Neill's Non-Hodkin Lymphoma
- Actor Sam Neil revealed his stage 3 blood cancer diagnosis, saying he is fighting angioimmunoblastic T-cell lymphoma (AITL).
- According to the National Organization for Rare Diseases, angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkin lymphoma.
- All non-Hodgkin lymphomas begin in white blood cells known as lymphocytes, which are part of your body’s immune system.
- From there, doctors separate these cancers into types depending on the specific kind of lymphocytes they grow from: B cells or T cells.
- Thankfully, advancements in lymphoma treatment are underway, including CAR T-cell therapy as a treatment for certain adult patients with large B-cell lymphoma (LBCL) that comes back.
The 75-year-old actor revealed his stage three blood cancer diagnosis to The Guardian this week, noting his specific disease is called angioimmunoblastic T-cell lymphoma (AITL).Read More
“I can’t pretend that the last year hasn’t had its dark moments. But those dark moments throw the light into sharp relief, you know, and have made me grateful for every day and immensely grateful for all my friends. Just pleased to be alive,” Neill told The Guardian.
He also admits his sickness forms a “spiral thread” throughout the narrative of his new memoir, “Did I Ever Tell You This?”. In fact, in the first chapter, he writes, “The thing is, I’m crook. Possibly dying. I may have to speed this up.”
“I found myself with nothing to do,” he told The Guardian. “And I’m used to working. I love working. I love going to work. I love being with people every day and enjoying human company and friendship and all these things. And suddenly I was deprived of that. And I thought, ‘what am I going to do?’”
He continued, “I never had any intention to write a book. But as I went on and kept writing, I realized it was actually sort of giving me a reason to live and I would go to bed thinking, ‘I’ll write about that tomorrow … that will entertain me.’ And so it was a lifesaver really, because I couldn’t have gone through that with nothing to do, you know.”
Neill’s memoir is set to be published on March 21, 2023.
What Is Angioimmunoblastic T-cell Lymphoma?
According to the National Organization for Rare Diseases, Angioimmunoblastic T-cell lymphoma (AITL) is a rare form of non-Hodgkin lymphoma.
Non-Hodgkin lymphoma is a “group of related malignancies (cancers) that affect the lymphatic system (lymphomas)” and lymphomas “are cancer of white blood cells (lymphocytes) and can be divided depending on the type of cells, B-lymphocytes (B-cells) or T-lymphocytes (T-cells).”
Angioimmunoblastic T-cell Lymphoma is classified as a Peripheral T-cell lymphoma and makes up 4% of all lymphomas.
“It is more common in older adults. It tends to involve the lymph nodes and bone marrow as well as the spleen or liver, which can become enlarged,” the American Cancer Society explains. “People with this lymphoma usually have fever, weight loss, and skin rashes and often develop infections.”
This type of lymphoma also has a quick progression. And although treatment is usually effective at the start, the lymphoma often is recurrent.
Learning about Non-Hodgkin Lymphoma
Non-Hodgkin Lymphoma: It’s More Than Just One Type
When people are diagnosed with non-Hodgkin lymphoma, they’re not talking about one type of cancer, but many. “Non-Hodgkin lymphoma is a big category,” Dr. Julie Vose, chief of hematology/oncology at the University of Nebraska Medical Center, previously told SurvivorNet.
All non-Hodgkin lymphomas begin in white blood cells known as lymphocytes, which are part of your body’s immune system. From there, doctors separate these cancers into types depending on the specific kind of lymphocytes they grow from: B cells or T cells.
Knowing whether you have a B-cell or a T-cell lymphoma is necessary as it will determine what kind of treatment is needed.
B-Cell Lymphomas Vs. T-Cell Lymphomas
B cells make proteins called antibodies that protect your body against bacteria, viruses, and other germs. Germs have a protein called an antigen on their surface, to which the antibody attaches. That signals your immune system to attack the invading organism.
If you live in the United States and are diagnosed with non-Hodgkin lymphoma, there’s a good chance it’s the B-cell variety. About 85% of all lymphomas diagnosed in North America are B-cell lymphomas. And in the United States, these cancers affect white people more often than African Americans or Asian people.
Other types of B-cell lymphomas are Follicular lymphoma, Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL), Mantle cell lymphoma, Marginal zone lymphomas, and Burkitt lymphoma.
T-cell lymphomas are more common in other parts of the world, such as Asia. That may have to do with causes that are unique to those regions, Dr. Vose says.
Do You Know Your B Cells From Your T Cells?
As for the T-cell lymphomas, the other category of non-Hodgkin lymphomas, these types of lymphomas are far less common than B-cell cancers. T cells either destroy germs themselves or increase the activity of other immune cells.
T-cell lymphomas, which are pretty rare and come in different types, affect only approximately 15% of people with non-Hodgkin lymphoma.
T-lymphoblastic lymphoma/leukemia affects mostly teens and young adults. This type of lymphoma grows quickly but is very curable.
Peripheral T-cell lymphomas, the type Neill has been diagnosed with, are a group of cancers that develop in mature T cells. Depending on the type, they can affect a variety of organs, including the skin, lungs, and intestines. Some of these cancers grow faster than others.
“B cells and T cells are both types of lymphocytes. Lymphocytes are part of your immune system,” Dr. Elise Chong, medical oncologist at Penn Medicine, previously told SurvivorNet. Lymphocytes are a type of white blood cell that helps your body fight infections.
Deciphering which lymphoma type you have, which your doctor will figure out through a biopsy, will ensure that you get the right treatment. Chemotherapy may be included as a treatment for both B-cell and T-cell lymphomas, however, other treatments are more specific to the cell type. For instance, the immunotherapy drug rituximab (Rituxan) only works against the CD20 antigen, which is on the surface of some B cells.
Prior to starting treatment, make sure you understand what kind of lymphoma you have, and what therapies are likely to be most effective against it.
In general, lymphoma treatment largely depends on the nature of your specific diagnosis. For non-Hodgkin lymphoma patients, their cancer is more likely to spread in a random way and be discovered in different groups of lymph nodes in the body. Hodgkin lymphoma cancers, on the other hand, are more likely to grow in a consistent way from one group of lymph nodes directly to another.
And even if you receive a lymphoma diagnosis at a later stage, Dr. Chong assured SurvivorNet that “unlike other cancers, where advanced stage is a death sentence, that’s certainly not the case for lymphoma.”
“We have many treatments with which people can either be cured with advanced stage lymphoma or have very good remissions,” Dr. Chong said. “So it doesn’t change how treatable someone is, even when they do have advanced stage lymphoma.”
Some lymphomas, for example, indolent lymphomas, may not even need to be treated immediately due to how slow they grow. In this case, careful monitoring – including imaging scans such as PET/CT – is used to monitor the progress of the cancer and access if treatment is needed yet.
“Where I use PET/CT in my practice quite a bit is if I’m observing a patient … and there is some new symptom or situation which makes me concerned that the patient may be changing from an indolent lymphoma to a more aggressive lymphoma,” Dr. Jakub Svoboda, a medical oncologist at Penn Medicine, told SurvivorNet in an earlier interview. “We refer to it as transformation.”
Advances in Lymphoma Treatment
Thankfully, advancements in lymphoma treatment are underway. According to the National Cancer Institute, most research non-Hodgkin lymphoma treatment is now focused on targeted therapy and immunotherapy, and researchers are also attempting to identify gene changes in different types of lymphoma that might be targeted for new drug development.
An impressive development from earlier this year was the U.S. Food and Drug Administration’s approval of Yescarta (axicabtagene ciloleucel) CAR T-cell therapy as a treatment for certain adult patients with large B-cell lymphoma (LBCL) that comes back.
CAR T-cell therapy stands for chimeric antigen receptor T-cell therapy, and it is a type of immunotherapy that uses your own immune cells to beat cancer.
Yescarta had previously been approved for LBCL patients who’ve had their second relapse, but the April 2022 approval meant that patients who went through their first relapse within one year or didn’t respond to treatment at all could use the drug. Additionally, the drug was approved for adult patients with large B-cell lymphoma that has stopped responding to first-line chemo-immunotherapy or relapsed within 12 months of first-line chemo-immunotherapy. First-line therapy is the first treatment given for a disease.
Dr. Alison Sehgal, a study investigator for the landmark clinical trial that the approval was based on, previously talked with SurvivorNet about what the expanding availability of this drug could do for patients.
“(The current standard of care) doesn’t work very well in that population, and that population is a pretty big group of people, unfortunately,” Dr. Sehgal, a hematologist and medical oncologist at UPMC Hillman Cancer Center in Pittsburgh, Penn., told SurvivorNet. “This is really practice-changing for a big portion of people with large B-cell lymphoma.”
RELATED: Study Finds The Drug Ibrutinib Can Improve Outcomes In Some Newly Diagnosed Patients With Mantle Cell Lymphoma
Ibrutinib is also currently being used and tested for lymphoma patients in numerous ways. This drug was created to stop the B-cell receptor signaling pathway that drives many types of non-Hodgkin lymphoma.
At the annual conference of the American Society of Clinical Oncology, an important new study learned that ibrutinib showed some effectiveness in fighting a type of non-Hodgkin lymphoma known as mantle cell lymphoma (MCL) – however, it has not yet increased the overall survival rate for patients with this disease.
As per the data from the randomized, phase 3 SHINE trial, using ibrutinib along with the standard of care regimen can improve progression-free survival, or PFS, by 2.3 years among newly diagnosed patients with MCL. PFS is the length of time from random assignment to disease progression or death. There was also higher toxicity noted with the three-drug regimen that included ibrutinib.
“It is still too soon to say if this regimen will lead to a new standard of care for older patients with mantle cell lymphoma,” Dr. Bijal Shah, a medical oncologist in Moffitt Cancer Center’s Malignant Hematology Department, told SurvivorNet. “I think a major challenge with the data presented relates to the need and benefit versus the toxicity of adding bendamustine.
“Notably, overall survival was not improved, and it suggests that a safe alternative is to deliver ibrutinib-based therapy at the time of relapse. It is hoped with future BTKi trials, we will learn more about the importance, or lack thereof, of the bendamustine component,” Dr. Shah added.
According to the National Cancer Institute, the drug has been approved for the treatment of small lymphocytic lymphoma and Waldenstrom macroglobulinemia, both indolent non-Hodgkin lymphomas, in the last several years. It has also received approval for mantle cell lymphoma (which can be aggressive or indolent) and marginal zone lymphoma (indolent).
There are also plenty of other improvements being made and drugs being studied at this time. If you’ve been diagnosed with lymphoma, make sure to discuss all your treatment options with your doctor. And hesitate to obtain multiple opinions on what treatment path is best for you.
Contributing: SurvivorNet Staff, Laura Gesualdi-Gilmore, Dr. Sufana Shikdar
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