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Angioimmunoblastic T-cell Lymphoma (AITL)

Angioimmunoblastic T-cell lymphoma (AITL) is a rare, aggressive blood cancer that affects your T cells (a type of white blood cell). Symptoms may include fever, skin rash and painless swelling in your neck, armpit or groin. Common treatments are chemotherapy drugs with steroids and stem cell transplants. Diagnosis usually happens in later stages.

Overview

What is angioimmunoblastic T-cell lymphoma (AITL)?

Angioimmunoblastic T-cell lymphoma (AITL) is a rare, fast-growing blood cancer that affects your T lymphocytes (T cells), a type of white blood cell. It can also spread to other areas of your body like your liver, lungs or bone marrow. AITL sits under the umbrella of non-Hodgkin T-cell lymphoma and is a subtype of peripheral T-cell lymphoma.

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Typically, T lymphocytes destroy harmful microorganisms like viruses and bacteria. Under ordinary circumstances, they can also kill cancer cells. But sometimes, a T lymphocyte can transform into a cancer cell. More T cells may follow suit and spread uncontrollably. That’s how AITL happens.

How common is angioimmunoblastic T-cell lymphoma?

While AITL is one of the more common subtypes of T-cell lymphoma, it’s rare overall. In the United States, AITL accounts for about 4% of all lymphoma cases and 1% to 2% of all non-Hodgkin lymphoma cases. It’s most common in people over the age of 65.

Symptoms and Causes

What are the symptoms of angioimmunoblastic T-cell lymphoma?

AITL is a fast-growing cancer, and symptoms can develop quickly. Symptoms associated with AITL may include:

Because AITL attacks the lymphatic system (part of your immune system), many people with this type of cancer also develop autoimmune diseases like immune thrombocytopenia or autoimmune hemolytic anemia.

What causes angioimmunoblastic T-cell lymphoma?

AITL happens when your T cells become abnormal and grow out of control. Researchers aren’t sure why some people develop T-cell lymphomas and others don’t.

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There might be a link between AITL and viral infections like Epstein-Barr. But scientists need more information about why this connection exists.

Diagnosis and Tests

How is angioimmunoblastic T-cell lymphoma (AITL) diagnosed?

AITL is usually diagnosed with a lymph node biopsy. Healthcare providers suspect AITL if your tests results show abnormal T lymphocytes.

After healthcare providers confirm your diagnosis, an oncologist will do a physical examination and ask about your symptoms. They’ll also review your health history, including any current or previous health conditions.

Your healthcare provider will run some tests, which may include:

They’ll also order imaging tests like:

Diagnosing AITL (and all lymphomas) is difficult. In addition to its complex nature, symptoms can come on gradually and may mimic other conditions. For these reasons, most people with angioimmunoblastic T-cell lymphoma receive their diagnosis during the late stages.

Researchers continue looking for ways to diagnose AITL and other lymphomas earlier.

Management and Treatment

How is angioimmunoblastic T-cell lymphoma treated?

AITL treatment depends on many factors, like your symptoms, severity of disease and overall health. Treatment options for angioimmunoblastic T-cell lymphoma include:

  • Chemotherapy. Healthcare providers use a combination of chemotherapy drugs and steroids. The commonly used regimens are CHOP and BV+CHP. The acronym CHOP stands for the drugs used in this regimen: Cytoxan® (cyclophosphamide), hydroxydaunorubicin, Oncovin (vincristine sulfate) and prednisone. BV+CHP stands for brentuximab vedotin, cyclophosphamide, doxorubicin and prednisone.
  • Stem cell transplant. This treatment replaces cancerous stem cells with healthy ones. Your provider will either use your own healthy stem cells (autologous) or donated stem cells from another person (allogenic).
  • Targeted therapy. This treatment focuses on genetic mutations (changes) that turn healthy cells into cancer cells.

Prevention

Can AITL be prevented?

Most people with AITL have no preventable risk factors. There’s no way to prevent AITL or other types of lymphoma. Like most cancers, it’s just something that happens — and it doesn’t mean you’ve done anything wrong.

How can I reduce my risk?

While you can’t prevent angioimmunoblastic T-cell lymphoma, you can take steps to maintain a healthy immune system:

  • Eat lots of fruits, vegetables, whole grains and lean proteins.
  • Maintain regular physical activity.
  • Get quality sleep.
  • Maintain a weight that’s healthy for you.
  • Don’t vape or use tobacco products, including cigarettes and smokeless tobacco (like chewing tobacco or snuff).
  • Limit drinks containing alcohol.

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Outlook / Prognosis

How long can you live with angioimmunoblastic T-cell lymphoma?

AITL is an aggressive cancer. According to research, median survival after diagnosis is fewer than three years. (“Median” is the exact middle number in a list of values.) However, in approximately 30% to 35% of the people who have AITL, the lymphoma may not come back after responding to treatment.

AITL survival rate

The five-year survival rate for angioimmunoblastic T-cell lymphoma is 30% to 35%. That means that 30 to 35 out of 100 people with AITL are still alive five years after their diagnosis. Comparatively, the seven-year survival rate for AITL is 29%.

Interpreting survival rates can feel stressful and overwhelming. Remember, these numbers are only estimates. And they’re based on the prior experiences of other people with the same diagnosis. Each person has a different health history with unique genetics at play. That’s why survival rates can’t tell you how long you’ll live or how well you’ll respond to treatment.

If you’re worried about survival rates — or wondering what they mean for you — talk to your healthcare provider. They’re here to help you understand your diagnosis and connect you with any necessary resources.

Living With

When should I see my healthcare provider?

You should tell your healthcare provider any time you develop sudden symptoms like a skin rash, swelling in your lymph nodes, high fever or losing weight without trying.

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If you’re already undergoing treatment for AITL, call your oncologist right away if you develop any new symptoms or if your symptoms suddenly worsen. Due to the nature of this cancer, it’s important to begin treatment immediately.

AITL affects everyone differently. Your healthcare provider is the only person who can explain what your diagnosis means for you. Some people don’t want to know specifics, and that’s OK, too. You’re in charge of your care. And only you know what’s best for your mental, emotional and spiritual health.

What questions should I ask my doctor?

You probably have a lot of questions after an AITL diagnosis. Here are some to ask your oncologist:

  • How advanced is my cancer?
  • Has the cancer spread to any of my organs?
  • What are my treatment options?
  • How soon do I need to begin treatment?
  • Will I be able to work while I have treatment?
  • What kind of support or assistance am I likely to need during treatment?
  • Can you connect me with resources or support groups?
  • How can I take care of my mental health?

A note from Cleveland Clinic

A cancer diagnosis can come with a range of tough emotions, from sadness and anxiety to anger and rage. And because angioimmunoblastic T-cell lymphoma (AITL) is so rare, you might feel isolated — like no one really understands what you’re going through. But your healthcare team is here to help. They can recommend resources and support groups where you can meet others who are dealing with similar situations. You don’t have to do this on your own. Lean on the support of your loved ones and healthcare providers.

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Medically Reviewed

Last reviewed on 08/20/2024.

Learn more about the Health Library and our editorial process.

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