Anaplastic Large Cell Lymphoma

Anaplastic Large Cell Lymphoma (ALCL) is a rare type of cancer that affects white blood cells called lymphocytes, specifically T-cells or natural killer (NK) cells. It is a form of non-Hodgkin lymphoma and can appear in different parts of the body, including lymph nodes, skin, bone, or soft tissues. Early diagnosis and treatment are important for managing this condition.

What is Anaplastic Large Cell Lymphoma?

Anaplastic Large Cell Lymphoma (ALCL) is a rare cancer of the lymphatic system, which is part of your body's immune system. This type of cancer involves abnormal growth of T-cells or natural killer (NK) cells, which are types of white blood cells. ALCL is classified as a non-Hodgkin lymphoma and can be either systemic, affecting multiple parts of the body, or cutaneous, primarily affecting the skin.

Your lymphatic system includes a network of vessels, tissues, and organs like lymph nodes, spleen, and bone marrow. Lymphocytes, which are a type of white blood cell, help fight infections. In ALCL, these lymphocytes grow abnormally and can form tumors. There are different types of ALCL. Systemic ALCL (sALCL) is more aggressive and can spread throughout the body. Cutaneous ALCL (cALCL) mainly affects the skin and is generally less aggressive. Another specific type is breast implant-associated ALCL (BIA-ALCL), which develops in the fluid or capsule around breast implants. Many cases of systemic ALCL involve a specific genetic change called an ALK gene rearrangement. This change helps doctors understand the disease and plan treatment. ALK-positive ALCL generally has a better outlook than ALK-negative ALCL.

Symptoms

Symptoms of Anaplastic Large Cell Lymphoma (ALCL) can vary depending on where the cancer is located. Common signs often include swollen, painless lymph nodes in areas like your neck, armpit, or groin. Other general symptoms, sometimes called B symptoms, may include unexplained fever, drenching night sweats, and significant weight loss.

For systemic ALCL, the most common symptom is the enlargement of lymph nodes, which are small, bean-shaped glands that filter harmful substances from your body. These swollen lymph nodes are usually painless. Beyond swollen lymph nodes, people with systemic ALCL may experience a group of general symptoms known as B symptoms. These include fevers that come and go without an infection, heavy sweating at night that soaks your clothes, and losing more than 10% of your body weight over six months without trying. If the ALCL primarily affects the skin (cutaneous ALCL), you might notice skin lesions, such as red, raised bumps or ulcers. Other possible symptoms, depending on where the lymphoma grows, can include fatigue, loss of appetite, or pain in affected areas like bones or soft tissues.

Causes & risk factors

The exact cause of Anaplastic Large Cell Lymphoma (ALCL) is not fully understood, but it is not contagious. In many cases of systemic ALCL, a specific genetic change involving the ALK gene is found, which leads to abnormal cell growth. Certain types of ALCL, like breast implant-associated ALCL, are linked to specific risk factors.

Most cases of systemic ALCL are associated with a gene rearrangement called ALK translocation. This means a piece of one chromosome breaks off and attaches to another, creating a new gene that promotes uncontrolled cell growth. This genetic change is not inherited; it happens during a person's lifetime. While the precise reasons for these genetic changes are unknown, ALCL is not considered contagious and does not typically run in families. It can affect people of any age, though systemic ALCL is more common in children and young adults, while cutaneous ALCL tends to affect older adults. Breast implant-associated ALCL (BIA-ALCL) is a rare type of ALCL that develops in the fluid or scar tissue around breast implants. The risk is higher with textured implants compared to smooth ones. This specific type of ALCL is thought to be related to chronic inflammation around the implant, but it is not breast cancer itself.

How it's diagnosed

Diagnosing Anaplastic Large Cell Lymphoma (ALCL) typically involves a physical exam and several tests, with a biopsy being the most crucial step. A biopsy means taking a small tissue sample, usually from a swollen lymph node or skin lesion, to be examined under a microscope. Imaging tests and blood work also help determine the extent of the disease.

Your doctor will start with a physical exam to check for swollen lymph nodes and other signs. Blood tests can provide general information about your health, but they cannot diagnose ALCL directly. Imaging tests, such as CT scans (computed tomography) or PET scans (positron emission tomography), help doctors see where the lymphoma might be in your body and if it has spread. The definitive diagnosis of ALCL requires a biopsy. During a biopsy, a surgeon removes all or part of a suspicious lymph node or a piece of a skin lesion. A pathologist, a doctor who specializes in examining tissues, then studies the sample under a microscope to identify the specific type of lymphoma cells. Further tests on the biopsy sample, such as immunohistochemistry and genetic testing, are performed to look for specific markers like the ALK protein. These tests help confirm the diagnosis of ALCL and determine if it is ALK-positive or ALK-negative, which guides treatment decisions. A bone marrow biopsy may also be done to check if the lymphoma has spread to the bone marrow.

Treatment options

Treatment for Anaplastic Large Cell Lymphoma (ALCL) depends on the type, stage, and whether the ALK gene is present. Chemotherapy is the primary treatment for systemic ALCL, often combined with other therapies. Targeted therapy and radiation therapy may also be used, and in some cases, a stem cell transplant might be considered, especially if the lymphoma returns.

For systemic ALCL, chemotherapy is the main treatment. This involves using powerful drugs to kill cancer cells throughout the body. The specific chemotherapy regimen, which is a combination of drugs, will be chosen by your medical team based on your individual situation. Targeted therapy, such as brentuximab vedotin, is another important option. This type of drug specifically targets cancer cells while minimizing harm to healthy cells. It may be used as a first treatment or if the lymphoma comes back after initial chemotherapy. Radiation therapy, which uses high-energy rays to kill cancer cells, might be used for localized disease or to treat specific areas where the lymphoma is causing symptoms. In some cases, especially if systemic ALCL relapses (returns), a stem cell transplant (also called a bone marrow transplant) may be an option. This procedure replaces diseased bone marrow with healthy stem cells. For cutaneous ALCL, which is often less aggressive, treatment may involve radiation therapy, surgery to remove skin lesions, or topical treatments. Systemic treatments like chemotherapy are usually reserved for more widespread or persistent cutaneous ALCL.

Recovery & outlook

The recovery and outlook for Anaplastic Large Cell Lymphoma (ALCL) vary significantly depending on the type of ALCL, its stage, and whether it is ALK-positive or ALK-negative. ALK-positive systemic ALCL generally has a better prognosis than ALK-negative. Many people achieve remission, but regular follow-up care is essential to monitor for any recurrence.

For people with ALK-positive systemic ALCL, the outlook is generally favorable, with a high chance of achieving long-term remission, meaning the signs and symptoms of cancer disappear. However, ALK-negative systemic ALCL tends to be more challenging to treat and may have a less favorable prognosis. Cutaneous ALCL, which primarily affects the skin, often has an excellent prognosis and is typically less aggressive than systemic forms. It may even resolve on its own in some cases, though treatment is usually recommended to prevent recurrence. Even after successful treatment, there is a possibility that ALCL could return (relapse). Therefore, regular follow-up appointments with your healthcare team are crucial. These appointments typically include physical exams, blood tests, and imaging scans to monitor for any signs of the lymphoma returning. Your doctor will discuss a personalized follow-up plan with you.

When to see a doctor

You should see a doctor if you notice persistent or unexplained symptoms that could indicate Anaplastic Large Cell Lymphoma (ALCL). These include swollen lymph nodes that don't go away, especially in your neck, armpit, or groin, or if you experience unexplained fevers, drenching night sweats, or significant weight loss. Early medical evaluation is important for proper diagnosis.

It's important to remember that many common infections can cause swollen lymph nodes, and these symptoms do not always mean you have ALCL. However, if your lymph nodes remain swollen for several weeks, grow larger, or are accompanied by other concerning symptoms, it's wise to seek medical advice. Specifically, look out for the B symptoms: unexplained fevers, night sweats that soak your clothes, or losing a lot of weight without trying. If you experience any of these symptoms, especially in combination, contact your doctor promptly. They can perform an examination and recommend appropriate tests to determine the cause. If you have breast implants and notice any new or unusual swelling, pain, or a mass around the implant, you should also contact your doctor right away. While rare, these could be signs of breast implant-associated ALCL, which requires prompt medical attention.

Frequently asked questions

Is Anaplastic Large Cell Lymphoma (ALCL) contagious?

No, Anaplastic Large Cell Lymphoma (ALCL) is not contagious. It is a type of cancer that results from abnormal cell changes within an individual's body and cannot be spread from person to person.

What is the difference between ALK-positive and ALK-negative ALCL?

ALK-positive ALCL means the cancer cells have a specific genetic change involving the ALK gene. This type generally responds better to treatment and has a more favorable outlook than ALK-negative ALCL, which lacks this genetic change and can be more aggressive.

Can ALCL affect children?

Yes, systemic Anaplastic Large Cell Lymphoma (sALCL) is one of the more common types of non-Hodgkin lymphoma found in children and young adults. The ALK-positive form is particularly prevalent in pediatric cases.

What is breast implant-associated ALCL (BIA-ALCL)?

BIA-ALCL is a rare type of Anaplastic Large Cell Lymphoma that develops in the fluid or scar tissue surrounding breast implants. It is not breast cancer and is more commonly associated with textured breast implants.

How long does treatment for ALCL usually last?

The duration of ALCL treatment varies widely depending on the specific type of ALCL, its stage, and the chosen treatment plan. Chemotherapy regimens typically last several months, but follow-up care and monitoring can continue for years.

What happens if ALCL comes back after treatment?

If Anaplastic Large Cell Lymphoma (ALCL) returns after initial treatment, it is called a relapse. In such cases, your doctor will discuss further treatment options, which may include different chemotherapy drugs, targeted therapies, or a stem cell transplant.

Sources

• MedlinePlus — Anaplastic Large Cell Lymphoma
• Mayo Clinic — Anaplastic Large Cell Lymphoma
• Cochrane Library — Anaplastic Large Cell Lymphoma