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Actor Sam Neill discusses living with stage three angioimmunoblastic T-cell lymphoma (AITL) and his experimental treatment. Borja B. Hojas/Getty Images
  • Actor Sam Neill is battling stage 3 angioimmunoblastic T-cell lymphoma (AITL) and the experimental anticancer drug he is on will eventually stop working.
  • Symptoms of AITL include fever, weight loss, fatigue and enlarged lymph nodes.
  • AITL is caused by mutations in genes which lead to the development of cancer. It is most commonly seen in men over 60.
  • Chemotherapy is the most common treatment for AITL.

Actor Sam Neill has opened up about being diagnosed with a rare and often aggressive form of T-cell lymphoma.

Neill’s cancer is currently in remission with the help of an experimental drug. Neill opted for the drug after the cancer failed to respond to chemotherapy treatment.

The 76-year-old actor, most known for his roles in “Jurassic Park” and “The Piano,” told ABC’s “Australian Story” that the medication will eventually stop working. Neill said he is prepared for what’s to come and is “not remotely afraid” of death.

In a video posted to Instagram this week after the interview aired, Neill aimed to reassure fans that he is “firmly” in remission and hopes to remain so for years to come.

Sam Neill has stage 3 angioimmunoblastic T-cell lymphoma (AITL), which is a common type of T-cell lymphoma, but only comprises 1–2% of non-Hodgkins lymphomas, according to the Lymphoma Research Foundation.

“Angioimmunoblastic T-cell lymphoma (AITL) is a rare type of cancer that affects a type of white blood cells called T-cells and is considered a subtype of non-Hodgkin lymphoma,” Dr. Sara Taveras Alam, an oncologist with UTHealth Houston and Memorial Hermann, told Healthline.

“90% of patients present with an advanced stage and prognosis is generally poor with a 63% survival rate at 5 years at best. AITL is sensitive to chemotherapy and occasionally can be cured with a stem cell transplant.”

Most patients present with advanced-stage disease involving multiple lymph nodes and often the spleen and bone marrow.

According to Alam, symptoms of AITL can include:

  • fever
  • weight loss
  • fatigue
  • night drenching sweats
  • itching (pruritus) or rash
  • enlarged lymph nodes (for example in the neck, armpits or groin)
  • early satiety or enlarged abdomen (from enlarged liver and/or enlarged spleen)
  • autoimmune phenomena (where the body attacks itself)

“In some cases, the disease is difficult to diagnose as it can masquerade early on as vasculitis, arthritis, or other autoimmune disorders,” said Dr. Francine Foss, Hematology, Yale New Haven Health and professor of medicine, Medical Oncology, Yale Cancer Center, explained.

The causes of AITL are not well understood, but certain chronic conditions may play a role in the development of the disease. Thse include:

“AITL is caused by mutations in genes which lead to the development of cancer,” Foss explained.

“In the case of AITL, there are specific genes, called epigenetic regulatory genes, which are mutated and give rise to the disease. Recent studies using DNA sequencing have shown these mutations.”

Most cases of AITL are diagnosed in older males over 60, but the specific risk factors are not fully understood.

“The risk factors for most T-cell lymphomas are unknown,” said Foss. “What is known is that underlying autoimmune conditions or immunodeficiency syndromes, the use of immunosuppressive drugs, and reactivation of viruses such as the EBV virus can be associated with T-cell lymphomas.”

Chemotherapy is the most common treatment option for AITL.

One regimen called CHOP (cyclophosphamide doxorubicin, vincristine, and prednisone) is a combination of chemotherapy drugs commonly utilized for this disease, Alam stated.

Another option is CHOEP, with the addition of etoposide.

Corticosteroids like prednisone help reduce inflammation, manage symptoms and may also kill cancer cells.

Chemotherapy agents can be combined with a targeted therapy called brentuximab if a person has a certain gene marker on lymphoma cells, Alam added.

A stem cell transplant after high-dose chemotherapy may also be considered to increase the chance of cure.

“AITL is treated with aggressive chemotherapy,” said Foss.

“Newer approaches have included novel drugs which target proteins or the epigenetic mutations in the cells. Most patients with AITL are not cured with chemotherapy alone, and the recommendation is that patients undergo an autologous stem cell transplant with high dose chemotherapy and infusion of their own stem cells once they are in remission from chemotherapy,” Foss added.

A transplant has a cure rate of over 50% of patients. For those patients who cannot undergo transplants or who do not respond to chemotherapy, a number of clinical trials with novel agents and immunotherapies are currently underway.

In the case of AITL, understanding the biology of the disease by molecular sequencing and laboratory investigation has directly translated into novel more disease-focused treatments and hope for patients, Foss added.

Actor Sam Neill opened up about living with stage three angioimmunoblastic T-cell lymphoma (AITL) and the experimental anticancer drug he is on.

Fever, weight loss, fatigue and enlarged lymph nodes are several symptoms associated with AITL.

This type of lymphoma is caused by gene mutations. Most diagnoses are men over 60.

Chemotherapy is the most common course of treatment for AITL.