What is angioimmunoblastic T cell lymphoma The rare blood cancer that

What is angioimmunoblastic T-cell lymphoma? The rare blood cancer that affects 76-year-old Jurassic Park star Sam Neill affects just 5,000 Americans each year – but can kill 87% within 5 years

Sam Neill, the actor best known for his roles in Jurassic Park and Event Horizon, has reflected on his life and shared that he is “not afraid of dying from advanced blood cancer.”

Neill, 76, from New Zealand, announced earlier this year that he was battling stage three angioimmunoblastic T-cell lymphoma (AITL), a rare cancer that affects the lymphatic system, the body’s disease-fighting network.

After failed chemotherapy, Neill takes an experimental drug. However, he told TV show Australian Story that the drug wouldn’t last forever and he was in a “battle for life”.

“I’m not afraid of dying at all. That doesn’t worry me. “It never bothered me to begin with, but I would be annoyed,” he said.

“I would get annoyed because there are things I still want to do.” Very irritating, dying. But I’m not afraid of it.’

Below, answers questions about AITL, including how the disease ravages the body, what causes it and what the prognosis is.

Sam Neill suffers from 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 it to stop working at some point

Sam Neill suffers from 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 it to stop working at some point

Symptoms include persistent or recurrent fever, unexplained weight loss, night sweats, and enlarged lymph nodes in the neck, armpit, or groin.  A main symptom is a pronounced skin rash that may be red or characterized by small spots or bumps, similar to a measles rash

Symptoms include persistent or recurrent fever, unexplained weight loss, night sweats, and enlarged lymph nodes in the neck, armpit, or groin. A main symptom is a pronounced skin rash that may be red or characterized by small spots or bumps, similar to a measles rash

What is angioimmunoblastic T-cell lymphoma (AITL)?

AITL is a rare and aggressive form of non-Hodgkin’s lymphoma, a cancer of the lymphatic system. This is the body’s disease-fighting network, which consists of the spleen, bone marrow, lymph nodes and thymus.

The lymphatic system ensures a healthy fluid balance in the body by filtering out excess fluid and returning it to the bloodstream. According to the Cleveland Clinic, it also helps the body absorb fats and protect itself from bacteria, viruses, parasites and fungi.

AITL begins in T cells, a type of white blood cell – known as lymphocytes – that support healthy immune function by copying themselves to destroy incoming threats. AITL turns these T cells into malignant or cancerous cells.

According to the National Cancer Institute (NCI), it is considered an aggressive form of lymphoma.

What causes it?

Lymphomas occur when T cells mutate into cancer cells. Risk factors for lymphoma generally include previous infections such as human immunodeficiency virus (HIV) and Epstein-Barr virus, a family history, a weakened immune system due to taking immunosuppressants, and autoimmune diseases.

Some preliminary research suggests that overweight or obese adults are more likely to develop non-Hodgkin lymphoma.

Researchers aren’t sure what causes AITL.

How common does it occur?

The NCI estimates that there will be more than 80,000 cases of non-Hodgkin’s lymphoma and over 20,000 deaths in the United States this year.

AITL accounts for one to two percent of these cases. That’s about 5,000 Americans per year.

According to the Lymphoma Research Foundation, it is most common in older adults, especially people over the age of 70. Men are also more likely to develop non-Hodgkin lymphoma.

What are the symptoms?

The initial symptoms are difficult to recognize because they are similar to those of several other diseases. Researchers refer to these generalized symptoms as “B symptoms.”

According to the National Organization for Rare Disorders (NORD), these include persistent or recurrent fever, unexplained weight loss, night sweats, and enlarged lymph nodes in the neck, armpit, or groin.

A main symptom is a pronounced 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 being unwell called malaise, and fluid buildup in the lungs or abdomen.

What is the prognosis?

Sam Neill (left), Joseph Mazzello and Ariana Richards are pictured in 1993's Jurassic Park

Sam Neill (left), Joseph Mazzello and Ariana Richards are pictured in 1993’s Jurassic Park

The Lymphoma Research Foundation estimates that most patients with AITL are diagnosed at either stage three or four because early symptoms are difficult to detect.

The foundation determines survival rates based on a patient’s risk. Patients at higher risk tend to be older.

The survival rate for 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 disease progression-free survival rate of 41 percent and 63 percent overall.