There are two main types of lymphoma: Hodgkin and non-Hodgkin. SLL is a type of non-Hodgkin lymphoma. It’s a lot like chronic lymphocytic leukemia (CLL) and is treated the same way. The only difference between them is where they occur. When the cancer cells are mostly found in the lymph nodes, it’s called SLL. In the case of CLL, the cancer cells are generally located in the bloodstream and bone marrow, but they may also be present in the spleen and lymph nodes. For the cancer to be considered CLL, you must have at least 5,000 monoclonal lymphocytes (per cubic millimeter, or mm3) in your blood. For the cancer to be called SLL, you must have enlarged lymph nodes or an enlarged spleen with fewer than 5,000 lymphocytes (per mm3) in your blood. SLL and CLL are usually slow-growing and may not need to be treated immediately. These cancers primarily affect older people. (1,2,3) If you do experience symptoms, the first sign of SLL is usually swelling in the lymph nodes in the neck, armpit, or groin. Other symptoms may include:
FeverNight sweatsWeight lossLoss of appetiteFrequent infectionsFatigueShortness of breathEasy bruisingA swollen abdomenFeeling of fullness after only eating a small amount of food (4,5)
Other tests that may be used to help your doctor learn more about the cancer include:
A physical exam (to check for enlarged lymph nodes or an enlarged spleen)Blood testsX-raysImaging tests, such as a computerized tomography (CT) scanBone marrow sampling
Both CLL and SLL are managed and treated the same way. (6) Certain risk factors may raise your chances of developing the cancer. These include:
Being Older The most common age at which people are diagnosed with SLL is 65.Being Male Men are slightly more likely to get SLL and CLL than women.Having a Close Family Member With the Disease You’re a little more likely to develop lymphoma if you have a parent or sibling who had it.Having Other Medical Conditions Being diagnosed with rheumatoid arthritis, human immunodeficiency virus (HIV), Sjögren’s syndrome, lupus, or other cancers can increase your risk of developing lymphoma. Some viruses and infections can also up your odds. (7)
Staging lymphoma can help your physician recommend the most effective treatment approaches. Most types of non-Hodgkin lymphoma are staged with a system called the Lugano classification. It involves using the numbers 1 through 4 to categorize the cancer. If the lymphoma is a stage 1 or 2 but affects an organ outside the lymph system, it might also have a letter “E” added to the stage description. Here’s a breakdown of each stage:
Stage 1 The cancer is only found in one lymph node area or lymphoid organ.Stage 1E The lymphoma is found in only one area of a single organ outside the lymphatic system.Stage 2 The cancer is in two or more groups of lymph nodes on the same side of the diaphragm.Stage 2E The lymphoma is found in a group of lymph nodes and one area of a nearby organ. It might also be present in other groups of lymph nodes on the same side of the diaphragm.Stage 3 The cancer is found in lymph node areas on both sides of the diaphragm, or it’s in lymph nodes above the diaphragm and in the spleen.Stage 4 The lymphoma has spread to at least one organ outside the lymph system, such as the liver or lung.
If the cancer affects the blood or bone marrow, it’s often staged differently, using a system for CLL. (8)
Active Surveillance
If you have few or no symptoms, your doctor might decide not to treat the lymphoma right away. This is known as “active surveillance” or “watchful waiting.” Studies have shown that people with less advanced disease who follow an active surveillance approach have similar outcomes as those who are treated early on. With active surveillance, your physician will monitor the cancer and your overall health with regular checkups and tests. Treatment can be started if you develop symptoms or if tests show the cancer is progressing. (4)
Treatments
If your doctor recommends treatment, you may receive one medicine or a combination of different drugs. In some cases, these therapies may be given as part of a clinical trial. Or you might need a medical procedure to treat the cancer. Before starting any treatment, your doctor should do genetic tests on your cancer cells to identify specific abnormalities or mutations. This information can help your physician recommend the best treatment for you. Options for SLL generally include:
Chemotherapy Chemotherapy medicines can be given orally or through an injection into a vein. Some chemotherapy drugs for SLL are: cyclophosphamide (Cytoxan), fludarabine (Fludara), chlorambucil (Leukeran), pentostatin (Nipent), bendamustine (Bendeka), and lenalidomide (Revlimid). These drugs are often used in combination with each other or with another type of medicine.Monoclonal Antibodies These medicines use the body’s natural immune system to fight cancer. They’re typically administered by an injection and are often given along with chemotherapy or another type of drug. Monoclonal antibodies that are used for SLL may include: rituximab (Rituxan), alemtuzumab (Lemtrada), obinutuzumab (Gazyva), and ofatumumab (Arzerra).Targeted Treatments These therapies attack different targets on cancer cells. Ibrutinib (Imbruvica) and idelalisib (Zydelig) are examples of targeted treatments for SLL. Both of these medicines are pills that are taken by mouth and are sometimes used with other therapies. Other targeted treatments include acalabrutinib (Calquence), which may be used when the cancer has not responded to other drugs, and venetoclax (Venclexta), which is sometimes used for patients 75 years old or older or for adults who can’t be given standard chemotherapy drugs.Steroids Steroids may be used to destroy cancer cells or treat autoimmune issues in people with SLL or CLL.Radiation Radiation therapy uses high energy rays to destroy cancer cells. It’s occasionally used in people with SLL.Bone Marrow Transplant A bone marrow transplant, also called a “stem cell transplant,” may be an option for some people with SLL. It involves collecting stem cells from the blood or bone marrow of the patient or a matched donor. These cells are given back to the patient to restore damaged bone marrow after they receive high doses of chemotherapy, which wipe out the remaining bone marrow in their body. Because it can be hard on your body, a bone marrow transplant is often only performed on healthy, younger people.Splenectomy Surgery to remove the spleen doesn’t treat SLL itself, but some people might need to have it if they develop complications. (9,10,11)
Complications
SLL can hinder your immune system and make it more difficult for you to fight infections. Sometimes the disease causes your immune system to attack itself, and you may develop life-threatening conditions. Autoimmune hemolytic anemia (AIHA) happens when your immune system attacks your red blood cells. Immune thrombocytopenic purpura (ITP) occurs when your immune system targets the platelets in your blood. Both of these conditions may require immediate medical care. It is also possible for SLL or CLL to transform into a more aggressive form of lymphoma. (9)
Clinical Trials
Researchers are investigating many new therapies for SLL. If the disease is very advanced or doesn’t respond to current treatments, your doctor might recommend that you enroll in a study. Participating in a clinical trial may allow you to receive certain investigational therapies that aren’t yet available to the public. You can find out more about clinical trials by visiting the Leukemia and Lymphoma Society’s website or ClinicalTrials.gov.
Remission, Relapse, and Refractory Disease
After treatment for SLL, you may go into remission, which means there are no signs of cancer in your body. During this time, your doctor will continue to monitor your health. You might need to have certain tests, such as blood draws or CT scans, to periodically check for cancer. SLL often recurs, which means it returns after successful treatment. If this happens, you and your healthcare provider will come up with new treatment approaches. The word “refractory” means that the lymphoma doesn’t respond to treatments or the response doesn’t last very long. Your doctor might recommend different medicines or a clinical trial if the cancer become refractory. (12,13)
What stage is the lymphoma?Where is the cancer in my body?How fast is the lymphoma growing?Do you recommend treatment? Why or why not?What would happen if I didn’t have any treatment at all?What kind of side effects will the treatment cause?How can I stay healthy during treatment?Will I have to stop certain activities while receiving treatment?Are there any clinical trials I should consider?What is the outlook for this cancer?How will I know if the cancer relapses?What will my follow-up care involve?Where can I find support? (14)
Learning More About Cancer The American Cancer Society, the Leukemia and Lymphoma Society, and the Lymphoma Research Foundation offer credible information about lymphoma.Joining a Support Group In-person or online support groups can help you connect with others who have the same type of cancer.Exercising Being physically active may improve your health and make you feel better overall. Yoga and meditation can also reduce stress levels and make you feel calmer.Asking for Help Having a strong support system might help you cope with cancer. Ask family and friends for help when you need it. (15)
According to the National Cancer Institute, about 87.5 percent of people with SLL or CLL survive five or more years after diagnosis. (16) It’s important to know that survival statistics are only averages and can’t predict any one person’s outcome. Your prognosis will depend on several factors, including how advanced the cancer is, your overall health, and the treatments you’re given.
