Anaplastic large cell lymphoma

Overview

Anaplastic large cell lymphoma (ALCL) is a type of lymphoma. Lymphoma is cancer that affects the lymphatic system. The lymphatic system is made up of organs, glands, tubelike vessels and clusters of cells called lymph nodes. It's part of the body's germ-fighting immune system. Anaplastic large cell lymphoma can affect the lymph nodes, skin, breasts, liver, lungs, bones and other parts of the body.

There are many types of lymphoma. These types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. Anaplastic large cell lymphoma is a type of non-Hodgkin lymphoma. Anaplastic large cell lymphoma starts in germ-fighting white blood cells called T lymphocytes.

Anaplastic large cell lymphoma gets its name from the way the cancer cells look when viewed with a microscope. The cells are called large because they are much bigger than healthy cells. The cancer cells are called anaplastic because they look very different from healthy cells. They lose the round shape that healthy cells have. Also, the contents of the cells that are usually at the center often shift to the side.

Anaplastic large cell lymphoma is treatable and may be curable depending on the type. Treatments for anaplastic large cell lymphoma may include chemotherapy, radiation therapy, surgery, targeted therapy and bone marrow transplant, also called bone marrow stem cell transplant.

Types

Healthcare professionals classify anaplastic large cell lymphoma (ALCL) into several main types based on where it starts in the body and whether it makes a protein called anaplastic lymphoma kinase (ALK). The ALK protein is linked to how the lymphoma behaves and how it responds to treatment. The types of anaplastic large cell lymphoma include:

• Systemic ALK-positive ALCL. In this type, the cancer cells produce the ALK protein. Systemic ALK-positive ALCL mostly affects the lymph nodes, organs and skin. It is most common in children, teens and young adults. It often responds very well to treatment. People with ALK-positive ALCL usually have a better outlook than those with other types.
• Systemic ALK-negative ALCL. In this type, the cancer cells do not produce the ALK protein. This type mostly affects the lymph nodes, organs and skin. Systemic ALK-negative ALCL is more common in older adults. It may grow more quickly than other types or come back after treatment. Some people with this type have gene changes that can affect how well treatment works.
• Primary cutaneous ALCL. This type starts in the skin and is usually limited there. It causes bumps or lesions that may be red, purple or another color depending on skin color. These bumps or lesions may itch or form an open sore. Although it can come back after treatment, primary cutaneous ALCL usually grows slowly and rarely spreads to other parts of the body.
• Breast implant-associated (BIA) ALCL. This type develops around textured breast implants, often years after surgery. Breast implant-associated ALCL may cause swelling, pain or fluid buildup around the implant. The disease is found early in most people and can be treated successfully with surgery to remove the implant and nearby tissue.

Symptoms

Symptoms of anaplastic large cell lymphoma (ALCL) depend on the type and where it develops in the body.

Common signs and symptoms of ALK-positive and ALK-negative ALCL include:

• Swollen lymph nodes, which might feel like lumps under the skin. They happen most often in the neck, armpit and groin.
• Fever.
• Fatigue.
• Drenching night sweats.
• Weight loss that happens without trying.
• Pain or fullness in the abdomen.
• Itchy skin or a rash.

Symptoms of primary cutaneous ALCL may include:

• Itchy skin or rash.
• Nodules or sores on the upper half of the body.
• Patches or lesions on the skin.

Symptoms of breast implant-associated (BIA) ALCL may not happen until years after the implant surgery. Symptoms may include:

• A lump in the breast.
• Swollen lymph nodes in the armpit.
• Breast swelling or tenderness.
• Fluid buildup around the implant.

When to see a doctor

Make an appointment with a healthcare professional if you have any symptoms that worry you.

Causes

It's not clear what causes anaplastic large cell lymphoma (ALCL). Cancer happens when cells develop changes in their DNA. A cell's DNA holds the instructions that tell the cell what to do. In healthy cells, the DNA gives instructions to grow and multiply at a set rate. The instructions tell the cells to die at a set time. In cancer cells, the DNA changes give different instructions. The changes tell the cancer cells to make many more cells quickly. Cancer cells can keep living when healthy cells would die. This causes too many cells. In anaplastic large cell lymphoma, the cancer cells can build up in the lymph nodes, skin, breasts, liver, lungs, bones and other parts of the body.

Risk factors

Factors that increase the risk of anaplastic large cell lymphoma (ALCL) include:

• Your age. ALK-positive ALCL is more common in children and young adults. ALK-negative ALCL is more common in people over 50.
• Infection with human immunodeficiency virus (HIV). People with HIV have weakened immune systems. That increases the risk of ALK-negative ALCL.
• Ongoing inflammation. Inflammation caused by textured breast implants can increase the risk of breast implant-associated ALCL over time.

Complications

A potential complication of anaplastic large cell lymphoma (ALCL) is that the cancer may come back after treatment. This is called a relapse. Relapse is most common in people with ALK-negative ALCL. Relapse also is common in primary cutaneous ALCL.

Diagnosis

Diagnosis and tests

Diagnosis of anaplastic large cell lymphoma (ALCL) often begins with a physical exam. This exam checks for swollen lymph nodes in the neck, underarms and groin and an enlarged spleen or liver. Other tests and procedures include blood tests, imaging tests and taking a sample of tissue for lab testing.

Blood tests

Blood tests can sometimes show whether anaplastic large cell lymphoma cells are present. Blood tests may be used to test for viruses, including HIV, hepatitis B virus and hepatitis C virus. The presence of a virus may affect treatment options. Blood tests also measure levels of lactate dehydrogenase (LDH), which is often higher in people with lymphoma.

Imaging tests

Imaging tests make pictures of the body. They can show the location and extent of anaplastic large cell lymphoma. Tests might include MRI, CT and positron emission tomography (PET) scans. An ultrasound may be done on the breast to diagnose breast implant-associated ALCL.

Biopsy

Your healthcare professional may suggest a lymph node biopsy or a biopsy of other tissue to look for cancer cells. A biopsy is a procedure to remove a sample of tissue for testing in a lab. A lymph node biopsy involves removing all or part of a lymph node. A sample may be taken from the skin or other parts of the body depending on symptoms and imaging test results. A procedure called a fine-needle aspiration may be done if there is fluid around a breast implant. In the lab, tests may show whether you have anaplastic large cell lymphoma.

Bone marrow aspiration and biopsy

Bone marrow aspiration and biopsy are procedures to collect cells from the bone marrow for testing. Bone marrow is the soft matter inside bones where blood cells are made. Bone marrow has a solid part and a liquid part.

In a bone marrow aspiration, a needle is used to collect a sample of the fluid. In a bone marrow biopsy, a needle is used to collect a small amount of the solid tissue. Most often, the samples are taken from the hip bone. The samples go to a lab for testing. In anaplastic large cell lymphoma, this procedure can show whether the bone marrow is involved. It also can help find out the extent of the cancer.

Testing lymphoma cells in the lab

Lymphoma cells collected from a biopsy or a bone marrow aspiration and biopsy go to a lab for testing. In the lab, specialized tests look for specific things about the cells. The healthcare team uses the results to find out the type of anaplastic large cell lymphoma that you have.

To learn whether the cells are anaplastic large cell lymphoma cells, the healthcare professionals in the lab look for:

• Proteins on the surface of the cancer cells. Anaplastic large cell lymphoma cells have certain proteins on their surfaces that help identify them. These proteins are known as markers. These markers can help identify the type of anaplastic large cell lymphoma. For example, anaplastic lymphoma kinase (ALK) is a protein marker found in ALK-positive ALCL. CD30 is a marker found in all types of ALCL.
• Changes in the cancer cell DNA. Cancer happens when cells get changes in their DNA. Lab tests can show which DNA changes are present in the lymphoma cells.

Treatment

Treatments for anaplastic large cell lymphoma (ALCL) may include chemotherapy, radiation therapy, surgery, targeted therapy and bone marrow transplant, also called bone marrow stem cell transplant. Which treatment is best for you depends on the type of anaplastic large cell lymphoma you have and the extent of your cancer, called the stage. Your healthcare team also considers how quickly the cancer is growing, your overall health and what you prefer.

Treatments for the different types of ALCL are as follows:

• ALK-positive ALCL. Treatment usually starts with chemotherapy and targeted therapy. Other treatments may include radiation therapy and bone marrow transplant.
• ALK-negative ALCL. Treatment usually starts with chemotherapy and targeted therapy. Other treatments may include radiation therapy and bone marrow transplant.
• Primary cutaneous ALCL. Treatment may involve radiation therapy and surgery to remove the cancer. Targeted therapy can help if the cancer spreads or comes back after treatment.
• Breast implant-associated ALCL. Treatment usually starts with surgery to remove the implants. Other treatments may include chemotherapy, radiation therapy and targeted therapy.

Chemotherapy

Chemotherapy treats cancer with strong medicines. There are many chemotherapy medicines. Most chemotherapy medicines are given through a vein. Some come in pill form. You may take a combination of chemotherapy medicines. Chemotherapy also may be combined with radiation therapy or targeted therapy. Chemotherapy may be an option for cancer that comes back after treatment, called relapsed lymphoma, or when other treatments haven't worked, known as refractory lymphoma.

Radiation therapy

Radiation therapy for cancer is a treatment that uses powerful energy beams to kill cancer cells. The energy can come from X-rays, protons or other sources. During radiation therapy, you lie on a table while a machine moves around you. The machine directs radiation to precise points in your body.

Radiation therapy can help treat primary cutaneous ALCL if the cancer is in a single lesion or area. Radiation therapy also can help treat breast implant-associated ALCL.

Surgery

Surgery may be used for primary cutaneous ALCL to remove lesions. Radiation therapy may be used after surgery to help kill any remaining cancer cells.

Surgery also may be used for breast implant-associated ALCL to remove the cancer, implants and surrounding tissue. Radiation therapy, chemotherapy or targeted therapy may be used after surgery to help kill any remaining cancer cells.

Targeted therapy

Targeted therapy for cancer is a treatment that uses medicines that attack specific chemicals in cancer cells. By blocking these chemicals, targeted treatments can cause cancer cells to die.

For anaplastic large cell lymphoma, targeted therapy medicines can target a protein that the cells sometimes make. This protein is called anaplastic lymphoma kinase (ALK). If tests show that your cancer makes this protein, your healthcare team might recommend a medicine called an ALK inhibitor. ALK inhibitors block the action of the ALK protein and stop the cancer cells from multiplying. ALK inhibitors only work on ALK-positive cancers. Other targeted therapy medicines that target cancer cells in different ways may be options for other types of ALCL.

Targeted therapy may be combined with chemotherapy or other treatments. It may be an option for relapsed or refractory anaplastic large cell lymphoma. Targeted therapy also may be an option for ALCL that has spread to the lymph nodes and causes symptoms.

Bone marrow transplant

A bone marrow transplant, also called a bone marrow stem cell transplant, involves putting healthy bone marrow stem cells into the body. Stem cells can come from your own body, called an autologous transplant. Stem cells also can come from a donor, called an allogeneic transplant.

A bone marrow transplant may help treat relapsed or refractory ALK-positive ALCL and ALK-negative ALCL. Chemotherapy is typically done before the transplant to suppress the immune system and bone marrow.

Monitoring after treatment

After treatment is complete, you may have frequent follow-up appointments to see whether the cancer has come back, known as a relapse. You may have repeat blood and imaging tests and, if needed, biopsies, to check for a relapse.

Coping and support

With time, you'll likely find what helps you cope with the uncertainty and worry of a cancer diagnosis. Until then, you may find that it helps to:

Learn enough about anaplastic large cell lymphoma to make decisions about your care

Ask your healthcare team about your cancer, including your test results, treatment options and, if you want, your prognosis. As you learn more about anaplastic large cell lymphoma, you may become more confident in making treatment decisions.

Keep friends and family close

Keeping your close relationships strong can help you deal with anaplastic large cell lymphoma. Friends and family can provide the practical support you may need, such as helping take care of your home if you're in the hospital. And they can serve as emotional support when you feel overwhelmed by having cancer.

Find someone to talk with

Find someone who is willing to listen to you talk about your hopes and worries. This person may be a friend or family member. The concern and understanding of a counselor, medical social worker, clergy member or cancer support group also may be helpful.

Ask your healthcare team about support groups in your area, or contact organizations such as the American Cancer Society, Blood Cancer United and the Lymphoma Research Foundation. Find support online through Mayo Clinic Connect, which is a community where you can connect with others for support, practical information and answers to everyday questions.

Preparing for an appointment

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

If your healthcare professional thinks you might have anaplastic large cell lymphoma, you may be referred to a doctor who specializes in diseases that affect blood cells, called a hematologist. If a cancer diagnosis is made, you also may be referred to a doctor who specializes in treating cancer, called an oncologist.

Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.

What you can do

• Be aware of anything you need to do ahead of time. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down symptoms you have, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down important personal information, including major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements you're taking and the doses.
• Take a family member or friend along. It can be hard to remember all the information you get during an appointment. Someone who goes with you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

For anaplastic large cell lymphoma, some basic questions to ask include:

• Do I have anaplastic large cell lymphoma?
• What type of anaplastic large cell lymphoma do I have?
• What is the stage of my anaplastic large cell lymphoma?
• Has my anaplastic large cell lymphoma spread to other parts of my body?
• Will I need more tests?
• What are the treatment options?
• How much does each treatment prolong my life or increase my chances of a cure?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• Is there one treatment option you believe is the best?
• What would you recommend to a friend or family member in my situation?
• Should I see a specialist?
• Are there any brochures or other printed materials that I can take with me? What websites do you recommend?
• What will determine whether I should plan for a follow-up visit?

Don't hesitate to ask other questions.

What to expect from your doctor

Be prepared to answer questions, such as:

• When did your symptoms begin?
• Do your symptoms happen all the time or do you have them now and then?
• How bad are your symptoms?
• What, if anything, seems to make your symptoms better?
• What, if anything, seems to make your symptoms worse?
• Have you had any fevers, night sweats or weight loss?
• Have you noticed any lumps, swelling or pain anywhere in your body?
• What are your biggest worries about your diagnosis or treatment?