Adult T-cell leukemia/lymphoma (ATL)

Adult T-cell leukemia/lymphoma is a rare and aggressive type of blood cancer. It affects white blood cells called T-cells that normally help your immune system fight infections. When these cells become cancerous, they multiply out of control and can spread throughout your body.

ATL is caused by a virus called human T-lymphotropic virus type 1, or HTLV-I. Most people who have HTLV-I infection will never develop ATL. Only about 2 to 5 out of every 100 people with the virus develop this cancer, usually after decades of infection. The condition is more common in Japan, the Caribbean, parts of Africa, and South America where HTLV-I infection rates are higher.

There are four main types of ATL based on how quickly it progresses. Acute and lymphoma types grow very fast and require urgent treatment. Chronic and smoldering types develop more slowly. Understanding which type you have helps doctors plan the right treatment approach.

Symptoms of ATL vary depending on the type and how advanced the disease is. Common signs include:

• Swollen lymph nodes in the neck, armpits, or groin
• Frequent infections that are hard to shake
• Unexplained fever that comes and goes
• Night sweats that soak your clothes or sheets
• Extreme tiredness that does not improve with rest
• Unintentional weight loss of 10 pounds or more
• Skin rashes or lesions that may be itchy
• Bone pain or fractures from weakened bones
• Confusion or changes in mental function
• High calcium levels causing nausea or excessive thirst

Some people with slower-growing types of ATL may have no symptoms for months or years. Others may only notice swollen lymph nodes at first. Any persistent or concerning symptoms should be discussed with a doctor right away.

ATL is caused by long-term infection with the HTLV-I virus. This virus spreads through blood, sexual contact, breastfeeding, or sharing needles. Most people get infected during childhood through breastfeeding from an infected mother. The virus can stay dormant in your body for 20 to 60 years before ATL develops, if it develops at all.

Risk factors for developing ATL include being infected with HTLV-I at a young age, having a family history of the infection, and living in areas where the virus is common. People of Japanese or Caribbean descent have higher infection rates. Men develop ATL slightly more often than women. Having a weakened immune system may also increase your risk of the virus becoming active and causing cancer.

Diagnosing ATL requires several tests to confirm the presence of cancer and HTLV-I infection. Your doctor will start with a physical exam to check for swollen lymph nodes and an enlarged liver or spleen. Blood tests look for abnormal T-cells and check for HTLV-I DNA, which confirms infection with the virus. Additional blood work measures calcium levels, liver function, and how well your kidneys are working.

Your doctor may order a lymph node biopsy, where a small tissue sample is examined under a microscope. Imaging tests like CT scans or PET scans show how far the cancer has spread. Bone marrow biopsies help determine if cancer cells are present in your bone marrow. Talk to your doctor about which specialized tests are right for your situation and where to get them done.

Treatment for ATL depends on the type and how far the cancer has spread. Options include:

• Chemotherapy using multiple drugs to kill cancer cells
• Antiviral medications combined with immune system modulators
• Stem cell transplant for eligible patients with aggressive types
• Radiation therapy to shrink tumors in specific areas
• Antibiotics to prevent and treat infections
• Medications to lower high calcium levels
• Clinical trials testing new targeted therapies
• Supportive care to manage symptoms and improve quality of life
• Regular monitoring for slower-growing smoldering or chronic types

Lifestyle changes can help support your body during treatment. Eat a nutrient-rich diet with plenty of protein to maintain strength. Stay as active as possible to preserve muscle mass and energy. Avoid contact with people who are sick to reduce infection risk. Work closely with an oncologist who specializes in blood cancers to create a treatment plan that fits your needs.