Small lymphocytic lymphoma

Overview

Small lymphocytic lymphoma (SLL) 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.

There are many types of lymphoma. Lymphoma types are often divided into two categories: Hodgkin lymphoma and non-Hodgkin lymphoma. Small lymphocytic lymphoma is a type of non-Hodgkin lymphoma.

Small lymphocytic lymphoma can affect the lymph nodes, spleen, blood, bone marrow and other parts of the body. It usually grows slowly. Treatments usually can't cure small lymphocytic lymphoma, but they may control it for a long time. People with this type of cancer often have a good prognosis.

Not everyone needs treatment right away for small lymphocytic lymphoma. When needed, treatments for small lymphocytic lymphoma may include a "watch and wait" approach, targeted therapy, chemotherapy, immunotherapy and radiation therapy. Other treatment options may include bone marrow transplant, also known as bone marrow stem cell transplant, CAR-T cell therapy and clinical trials.

Some people wonder how small lymphocytic lymphoma relates to another disease called chronic lymphocytic leukemia. Healthcare professionals often mention these together because they are thought to be the same disease. However, in small lymphocytic lymphoma, the cancer cells are most often found in the lymph nodes. In chronic lymphocytic leukemia, the cancer cells are most often found in the blood and bone marrow.

Causes

It's not clear what causes small lymphocytic lymphoma. 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 small lymphocytic lymphoma, the cancer cells can build up in the lymph nodes, spleen, blood, bone marrow and other parts of the body.

Small lymphocytic lymphoma gets its name from the kind of cell that makes up this cancer. This cancer starts in germ-fighting white blood cells called lymphocytes. When healthcare professionals look at the cells with a microscope, the cancer cells appear round and small. These are sometimes called smudge cells.

Complications

Small lymphocytic lymphoma may cause complications such as:

• Frequent infections. If you have small lymphocytic lymphoma, you may get infections more often. These infections can be serious. Sometimes infections happen because your blood doesn't have enough germ-fighting antibodies, called immunoglobulins. Your healthcare professional might recommend regular immunoglobulin infusions.
• A switch to a more aggressive form of cancer. A small number of people with small lymphocytic lymphoma may develop a more aggressive form of cancer called diffuse large B-cell lymphoma. This is sometimes referred to as Richter syndrome.
• Increased risk of other cancers. People with small lymphocytic lymphoma have an increased risk of other types of cancer. These include skin cancer and cancers of the lung and the digestive tract.
• Immune system attacks. A small number of people with small lymphocytic lymphoma may develop an immune system reaction that causes the disease-fighting cells of the immune system to mistakenly attack the red blood cells. This is called autoimmune hemolytic anemia. The immune system also may attack the blood cells that help blood clot, called platelets. This attack is called autoimmune thrombocytopenia.

Treatment

Treatments for small lymphocytic lymphoma may include a "watch and wait" approach, targeted therapy, chemotherapy, immunotherapy and radiation therapy. Other treatment options may include bone marrow transplant, also known as bone marrow stem cell transplant, CAR-T cell therapy and clinical trials. Which treatment is right for you depends on whether you have symptoms 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.

Watch and wait

If your small lymphocytic lymphoma doesn't cause symptoms, you may not need treatment right away. Instead, you may have checkups every few months. The checkups help your healthcare team watch your condition to see if your cancer progresses.

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.

Targeted therapy is often the first treatment for small lymphocytic lymphoma. You may take a combination of targeted therapy medicines. Targeted therapy also may be combined with immunotherapy. Targeted therapy also may be used when other treatments haven't worked, known as refractory small lymphocytic lymphoma, or for cancer that comes back after treatment, called relapsed small lymphocytic lymphoma.

Side effects of targeted therapy may include diarrhea, liver issues, high blood pressure, and issues with blood clotting and wound healing. Side effects also may include fatigue, mouth sores and nail changes. Most side effects go away after treatment is done.

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.

Chemotherapy may be a treatment for small lymphocytic lymphoma. You may take several chemotherapy medicines at one time. Chemotherapy also may be combined with immunotherapy. Chemotherapy may be the first line treatment for older adults or others who can't tolerate targeted therapy. Chemotherapy also may be used for small lymphocytic lymphoma that has transformed into a more aggressive cancer.

Side effects of chemotherapy depend on the medicines you're given. Common side effects are nausea and hair loss. Serious long-term complications can happen, such as heart disease, lung damage, infertility and developing another kind of cancer, called a secondary cancer.

Immunotherapy

Immunotherapy for cancer is a treatment with medicine that helps the body's immune system kill cancer cells. The immune system fights off diseases by attacking germs and other cells that shouldn't be in the body. Cancer cells survive by hiding from the immune system. Immunotherapy helps the immune system cells find and kill the cancer cells.

Immunotherapy may be combined with targeted therapy or chemotherapy to treat small lymphocytic lymphoma. Immunotherapy also may be used for small lymphocytic lymphoma that has transformed into a more aggressive cancer.

Side effects of immunotherapy may include constipation, cough, fatigue, diarrhea, headache, muscle spasms and nausea.

Radiation therapy

Radiation therapy treats cancer with powerful energy beams. 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 is sometimes used to treat early-stage small lymphocytic lymphoma.

Radiation therapy side effects include fatigue and skin irritation at the site where the radiation is aimed. Other side effects depend on where the radiation is aimed. Radiation to the neck can cause dry mouth and damage the thyroid. Radiation to the chest can damage the heart and lungs.

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. These cells replace cells hurt by chemotherapy and other treatments. 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 be done for relapsed or refractory small lymphocytic lymphoma. A bone marrow transplant also may be an option for small lymphocytic lymphoma that has transformed into a more aggressive cancer. Chemotherapy is typically done before the transplant to suppress the immune system and bone marrow.

Short-term side effects of a bone marrow transplant may include upset stomach, vomiting and not feeling hungry. They also may include fatigue, mouth sores, hair loss and skin reactions. Long-term side effects may include infertility, secondary cancers, organ damage, weakness in the bones or muscles, and cataracts.

CAR-T cell therapy

Chimeric antigen receptor (CAR)-T cell therapy trains the immune system cells to fight lymphoma. This treatment begins with removing some white blood cells, including T cells, from the blood.

The cells are sent to a lab. In the lab, the cells are treated so that they make special receptors. The receptors help the cells recognize a marker on the surface of the lymphoma cells. Then the cells go back into the body. They find and destroy lymphoma cells.

CAR-T cell therapy may be an option for some people with refractory or relapsed small lymphocytic lymphoma. CAR-T cell therapy also may be an option for small lymphocytic lymphoma that has transformed into a more aggressive cancer. CAR-T cell therapy is typically used after at least two other treatments have been tried.

Side effects of CAR-T cell therapy may include fever, upset stomach, headache and confusion. They also may include dizziness, rash, rapid heartbeat, trouble breathing and low blood pressure.

Clinical trials

Clinical trials are studies of new treatments. These studies provide a chance to try the latest treatments. The risk of side effects might not be known. Ask your healthcare team if you can be in a clinical trial.

Monitoring after treatment

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

Treatment complications

Treatments for small lymphocytic lymphoma may have complications, including:

• Bone marrow suppression. When the bone marrow is suppressed, it can't produce enough blood cells, including white blood cells. White blood cells help fight infections. With a lower amount of white blood cells, you are at a higher risk of infections.
• Febrile neutropenia. Febrile neutropenia is a serious condition that can happen in people being treated for cancer. Febrile means having a fever. Neutropenia means having a low number of neutrophils, a type of white blood cell that helps fight infections. In febrile neutropenia, the body is trying to fight an infection but doesn't have enough neutrophils.
• Medicine toxicity. Some medicines used to treat cancer can be harmful to the body. They can cause organ damage and other issues. Whether a medicine is toxic depends on the type and how much you receive. There are many different medicine options. Your healthcare team can work with you to find the best medicines for you.
• Reactivating viruses. If you have had a viral infection in the past, such as hepatitis B or hepatitis C, some treatments can cause the virus to become active again. This can lead to liver inflammation, liver damage and other complications.
• Tumor lysis syndrome. Tumor lysis syndrome is a serious condition that can happen when cancer cells break down quickly after treatment or randomly. When these cells die, they release substances into the bloodstream that can overwhelm the body. This can lead to issues with the kidneys and other organs. You may take medicines to treat or prevent harmful side effects.

Preparing for an appointment

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

If your healthcare professional thinks you might have small lymphocytic lymphoma, you may be referred to a doctor who specializes in diseases of the blood and bone marrow, called a hematologist.

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, 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 small lymphocytic lymphoma, some basic questions include:

• Do I have small lymphocytic lymphoma?
• What is the stage of my small lymphocytic lymphoma?
• Will I need more tests?
• What are the treatment options?
• How much does each treatment prolong my life?
• What are the potential side effects of each treatment?
• How will each treatment affect my daily life?
• What treatment options have shown the best results?
• 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 material 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 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?

If you're diagnosed with small lymphocytic lymphoma, the next step is to determine the cancer's extent, called the stage. Imaging tests, blood tests and biopsies may be used to determine the stage of small lymphocytic lymphoma. Your healthcare team uses the cancer stage to help create your treatment plan.

The stages of small lymphocytic lymphoma range from 1 to 4:

• Stage 1 small lymphocytic lymphoma. At this stage, only one lymph node region or one site outside of the lymph nodes is affected.
• Stage 2 small lymphocytic lymphoma. Stage 2 involves two or more lymph node regions on the same side of the diaphragm or one lymph node region and a site outside of the lymph nodes.
• Stage 3 small lymphocytic lymphoma. At stage 3, lymph node regions on both sides of the diaphragm or lymph nodes above the diaphragm and the spleen are affected.
• Stage 4 small lymphocytic lymphoma. At stage 4, small lymphocytic lymphoma involves one or more organs including the liver, lungs and bones, and may or may not include the lymph nodes.

Survival rates are different for each person with small lymphocytic lymphoma. Healthcare professionals use something called prognostic risk categories. These categories group people into different levels of risk to show how the disease might act and what survival chances look like. Prognostic risk categories for small lymphocytic lymphoma include low, intermediate, high and very high. If you are in the low-risk group, your cancer is likely slow-growing, and your chances of living longer are better. If you are in the very high-risk group, your cancer is more serious, and your survival chances are lower. Your risk category is determined by:

• Your age.
• Whether certain gene changes are present in your cancer.
• How much of a specific protein is in your blood.
• The stage of your cancer.

For example, someone in the low-risk category for small lymphocytic lymphoma has a five-year survival rate of about 93%. As the risk gets higher, the survival rate gets lower. The five-year survival rate for someone in the very high-risk category for small lymphocytic lymphoma is about 23%.

Keep in mind that survival statistics take five years to collect. The most recent survival rates include people who had treatment for small lymphocytic lymphoma more than five years ago. These people may not have had access to the latest treatments. Over the last few decades, small lymphocytic lymphoma death rates have been falling and survival rates have been increasing.