Sam Neill, the actor best known for his roles in Jurassic Park and Event Horizon, reflected on his life and shared that he is “not afraid of dying” from advanced blood cancer.
Neill, 76, of New Zealand, announced earlier this year that he is fighting stage three angioimmunoblastic T-cell lymphoma (AITL), a rare cancer that affects the lymphatic system, the body’s network that fights diseases.
After a failed course of chemotherapy, Neill is taking an experimental drug. However, he told TV show Australian Story the drug won’t last forever, leaving him in a “fight for life”.
“I have no fear of dying. That doesn’t worry me. It never worried me from the beginning, but it would make me angry,” he said.
‘I would be angry because there are things I still want to do. Very irritating, dying. But I’m not afraid of him.’
Below, DailyMail.com answers questions about AITL, including how the disease wreaks havoc on the body, what causes it, and what the prognosis is.
Sam Neill has stage three angioimmunoblastic T-cell lymphoma (AITL) and has been in remission for 12 months thanks to a cancer drug, but admits he is “prepared” for the fact that it will eventually stop working.
Sam Neill (left), Joseph Mazzello and Ariana Richards appear in 1993’s Jurassic Park.
What is angioimmunoblastic T-cell lymphoma (AITL)?
AITL is a rare and aggressive form of non-Hodgkin lymphoma, a cancer of the lymphatic system. This is the body’s disease-fighting network and is made up of the spleen, bone marrow, lymph nodes, and thymus.
The lymphatic system maintains healthy fluid levels in the body by filtering excess and returning it to the bloodstream. It also helps the body absorb fats and protect against bacteria, viruses, parasites and fungi, according to the Cleveland Clinic.
AITL begins in T cells, a type of white blood cell, known as a lymphocyte, that supports healthy immune function by copying itself to destroy incoming threats. AITL converts those T cells into malignant or cancerous cells.
It is considered an aggressive form of lymphoma, according to the National Cancer Institute (NCI).
What causes it?
Lymphoma occurs when T cells mutate into cancer cells. Risk factors for lymphoma, in general, include having had previous infections such as human immunodeficiency virus (HIV) and Epstein-Barr virus, family history, a weakened immune system from taking immunosuppressive medications, and autoimmune diseases.
Some preliminary research suggests that adults who are overweight or obese are more likely to get non-Hodgkin lymphoma.
Researchers are not sure what causes AITL.
How common is it?
The NCI estimates that there will be more than 80,000 cases of non-Hodgkin lymphoma in the United States this year, along with more than 20,000 deaths.
AITL accounts for one to two percent of these cases. This is equivalent to about 5,000 Americans a year.
It is more common in older adults, particularly those over 70, according to the Lymphoma Research Foundation. Men are also more likely to get non-Hodgkin lymphoma.
What are the symptoms?
The initial symptoms are difficult to detect as they mimic those of other conditions. Researchers refer to these generalized symptoms as “B symptoms.”
According to the National Organization for Rare Diseases (NORD), these include persistent or repeated fever, unexplained weight loss, night sweats, and enlarged lymph nodes in the neck, armpits, or groin.
A key symptom is a distinctive skin rash that may be red or characterized by small spots or bumps, similar to a measles rash.
People with AITL may also experience fatigue, a general feeling of ill health known as malaise, and fluid buildup around the lungs or abdomen.
What is the prognosis?
The Lymphoma Research Foundation estimates that most patients with AITL are diagnosed at stage three or four, since early symptoms are difficult to detect.
The foundation determines survival rates based on how high risk a patient is. Patients at highest risk tend to be older. The survival rate among high-risk patients after five years without disease progression is only 13 percent. Overall, the survival rate for these patients is 21 percent.
Low-risk patients have a progression-free survival rate of 41 percent and 63 percent overall.