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.

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.

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.

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.

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?

Survival rates

The chance of surviving anaplastic large cell lymphoma (ALCL) is different for every person. Your personal prognosis may depend on:

• Your age.
• Whether your cancer makes the anaplastic lymphoma kinase (ALK) protein.
• The type of anaplastic large cell lymphoma you have.
• Whether certain gene changes are present in your cancer.
• The stage of your cancer.

To understand anaplastic large cell lymphoma survival rates, experts study many people with ALCL to see how many are living five years after diagnosis.

• ALK-positive ALCL. Studies of the five-year overall survival rate for this type have found rates between 70% and 86%. Younger people often have excellent long-term survival. Older adults or people with advanced cancer have poorer survival rates.
• ALK-negative ALCL. Studies of the five-year overall survival rate for this type have found rates between 30% and 49%. With this type, the presence of certain gene changes can affect your prognosis.
• Primary cutaneous ALCL. Studies of the five-year overall survival rate for this type have found rates between 81% and 90%.
• Breast implant-associated ALCL. When treated early with surgery, the five-year survival rate is about 89%.

Keep in mind that survival statistics take five years to collect. The most recent survival rates include people who had anaplastic large cell lymphoma treatment more than five years ago. They may not have had access to the latest treatments.