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.