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Patient education: Chronic lymphocytic leukemia (CLL) in adults (Beyond the Basics)

Patient education: Chronic lymphocytic leukemia (CLL) in adults (Beyond the Basics)
Literature review current through: Jan 2024.
This topic last updated: Jan 10, 2024.

CHRONIC LYMPHOCYTIC LEUKEMIA OVERVIEW — Chronic lymphocytic leukemia (CLL) is a chronic (long-term, slowly developing) leukemia. Leukemia is a type of cancer that affects the blood and bone marrow. Bone marrow is the spongy, red tissue that fills the large bones. All of the blood cells (red blood cells, white blood cells, and platelets) are derived from stem cells in the bone marrow. CLL is one of a group of diseases that affects a type of white blood cell called a "lymphocyte." Normally, lymphocytes help your body fight infection; however, in CLL, the body produces abnormal lymphocytes that cannot do this effectively. This leads to problems over time.

Small lymphocytic lymphoma (SLL) is a variant of CLL. If you have been diagnosed with SLL, the information in this article applies to you, too.

In CLL, high numbers of abnormal lymphocytes are found in the blood, lymph nodes, spleen, and bone marrow. In SLL, these same cells are commonly found in the lymph nodes. The abnormal cells cannot fight infection as normal lymphocytes do, but instead build up in lymph nodes and other areas, such as the liver and spleen. The accumulation of ineffective lymphocytes can interfere with the production of other blood cells, such as red blood cells and platelets, as well as the immune system.

Unlike some other types of leukemia, CLL usually progresses slowly. In many cases, it causes few, if any, problems in its early stages. Some people can live with CLL for decades. Some people, however, do not live as long. Many times, it is diagnosed incidentally, by blood tests that are performed during a routine physical exam. In other cases, a person is diagnosed after noticing symptoms and seeking medical care. (See 'Symptoms of chronic lymphocytic leukemia' below.)

Your doctors will work with you to determine the best course of treatment based on your specific situation. Unlike many other types of cancer, people with early stage CLL do not benefit from early, aggressive treatment, but instead do better with careful long-term monitoring of the disease.

More detailed information about CLL, written for health care providers, is available by subscription. (See 'Professional level information' below.)

CANCER CARE DURING THE COVID-19 PANDEMIC — COVID-19 stands for "coronavirus disease 2019." It is an infection caused by a virus called SARS-CoV-2. The virus first appeared in late 2019 and has since spread throughout the world. Getting vaccinated lowers the risk of severe illness; experts recommend COVID-19 vaccination for anyone with cancer or a history of cancer.

In some cases, if you live in an area with a lot of cases of COVID-19, your doctor might suggest rescheduling or delaying medical appointments. But this decision must be balanced against the importance of getting care to screen for, monitor, and treat cancer. Your doctor can talk to you about whether to make any changes to your appointment schedule. They can also advise you on what to do if you test positive or were exposed to the virus.

SYMPTOMS OF CHRONIC LYMPHOCYTIC LEUKEMIA — As mentioned, most people do not have symptoms when they are diagnosed with CLL, and the disease is discovered after a routine blood test shows abnormalities. However, some people do experience symptoms before diagnosis. The most common symptom is enlarged lymph nodes, most often in the neck, armpit, or groin areas (figure 1).

Less commonly, people with CLL notice other symptoms, including fatigue, unintentional weight loss, chronic fever (without other signs of infection), or night sweats.

The decision to start treatment is impacted by whether or not there are symptoms. (See 'Who should get treatment?' below.)

STAGING OF CHRONIC LYMPHOCYTIC LEUKEMIA — The progression of CLL can vary considerably from one person to another. Some people become sick within a short time of diagnosis; others live comfortably for years without problems. Doctors use different information to help them determine which people are most likely to get sick, and therefore most likely to benefit from treatment.

"Staging" is the approach doctors use to determine how advanced a person's cancer is. It involves assigning a category based on the physical examination and blood test results.

Staging systems — There are two systems used for staging CLL.

The Rai system — The Rai system for staging CLL is based on an analysis of how the body is affected by the abnormal lymphocytes. The original system had five stages, which were subsequently organized into three "risk groups." The higher numbers indicate a more advanced stage of disease:

Low risk (Stage 0) – Increased numbers of abnormal lymphocytes are found in the blood or bone marrow, lymph nodes/organs are not swollen, and production of red blood cells and platelets is not significantly affected.

Intermediate risk (Stage I and II combined) – Increased abnormal lymphocytes with enlarged lymph nodes, liver, and/or spleen. The production of red blood cells and platelets is not significantly affected.

High risk (Stage III and IV combined) – Increased abnormal lymphocytes with a low red blood cell count (anemia) and/or a low platelet count, with or without an enlarged spleen, liver, or lymph nodes.

The Binet system — This approach considers the five possible sites where lymphocytes can collect (lymph nodes in the neck, armpit, and groin, and lymphocyte-containing channels in the spleen and liver) (figure 1), and also whether blood tests show anemia or low platelet counts. The Binet system categorizes a person's cancer into one of three stages:

Stage A – Fewer than three involved sites; red blood cells and platelets are not significantly affected

Stage B – Three or more involved sites; red blood cells and platelets are not significantly affected

Stage C – Presence of anemia and/or low platelet counts regardless of the number of involved sites

What the CLL stages mean — Staging CLL helps your doctors determine how likely it is that you will develop serious problems related to your illness. In either system, the lower numbers or letters indicate a lower level of risk, while the higher numbers or letters indicate a higher level. Staging your cancer is a critical part of determining whether you should start treatment right away or delay treatment while undergoing regular monitoring.

Who should get treatment? — As noted above, treatment is not always required for CLL. Some studies have shown that people without symptoms of CLL are no more likely to die than a person of the same sex around the same age who does not have CLL. (See 'Asymptomatic, early-stage CLL' below.)

However, there are certain groups of people in whom doctors generally recommend treatment. This includes people with:

Symptoms of anemia and/or low platelets (in particular, high-risk Rai stages [III or IV] or Binet stage C)

Disease-related symptoms such as severe fatigue, night sweats, unintentional weight loss, painful swelling of lymph nodes or spleen, or unexplained fever (see 'Symptoms of chronic lymphocytic leukemia' above)

Extremely enlarged lymph nodes or spleen

Complications from involvement of other organs (such as the skin, kidney, lung, or spine)

Autoimmune hemolytic anemia (a condition in which the immune system destroys healthy red blood cells) or immune thrombocytopenia (a condition in which the immune system destroys healthy platelets) that does not respond to specific treatment for these complications

Cancer that is progressing quickly, as demonstrated by rapidly increasing white cells in the blood and/or rapidly enlarging lymph nodes, spleen, or liver

Recurring infections

INITIAL MANAGEMENT OF CHRONIC LYMPHOCYTIC LEUKEMIA

Asymptomatic, early-stage CLL — If your CLL is not causing symptoms, your doctor will likely not start treatment right away. However, you will need to be monitored regularly with blood tests and a physical exam. This usually involves seeing your doctor at least once every three months for the first year after diagnosis. Depending on the results of these tests, your doctor may be able to estimate how aggressive your disease will be in the future.

Advanced or symptomatic CLL — If you have advanced or symptomatic CLL, your doctor will talk to you about your treatment options. The options available will depend on your overall health and whether your cancer has certain characteristics (ie, mutations) found on genetic testing. For example, CLL with 17p deletion (in which part of chromosome 17 is missing) or TP53 mutation (when a gene called TP53 is abnormal) is usually more aggressive. CLL with mutations in the IGHV genes tends to be less aggressive.

There are three main options for initial treatment. They differ significantly in their side effects and how you take them.

Bruton tyrosine kinase (BTK) inhibitors – These are oral medications (taken as pills) given until disease progression or unacceptable side effects. While side effects are greatest in the first six months of treatment, they continue for the duration of treatment. Important side effects include bleeding, atrial fibrillation (an irregular heart rhythm), and high blood pressure. Other side effects include fatigue, rash, infections, and muscle and joint pain. People with significant side effects may be able to tolerate a different BTK inhibitor. "Covalent" BTK inhibitors are used for the initial treatment of CLL; these include acalabrutinib (brand name: Calquence), zanubrutinib (brand name: Brukinsa), and ibrutinib (brand name: Imbruvica).

Venetoclax (brand name: Venclexta) plus obinutuzumab (brand name: Gazyva) – This is a more intensive therapy given over one year, followed by a treatment-free period. People with a prolonged treatment-free period can be retreated with a venetoclax-based regimen. Venetoclax is taken orally (in pill form) and obinutuzumab is given intravenously (through an IV). This is a very potent drug combination; people taking this combination are monitored closely for something called "tumor lysis syndrome" or "TLS." TLS is a medical emergency due to the rapid destruction of cancer cells and release of substances into the blood. When the cancer cells break down, this causes very high levels of potassium, phosphate, and uric acid in the body and very low levels of calcium. This can cause damage to the kidneys as well as other organs. Severe TLS is uncommon with proper monitoring. Common side effects include low blood counts, diarrhea, nausea, infections, fatigue, swelling, and musculoskeletal pain.

Ibrutinib (brand name: Imbruvica) plus venetoclax (brand name: Venclexta) – This is a more intensive therapy given over 15 months, followed by a treatment-free period. Availability varies as this combination has regulatory approval in Europe but not the United States. People with a prolonged treatment-free period can be retreated with a venetoclax-based regimen. Both ibrutinib and venetoclax are taken orally (in pill form). This is also a very potent drug combination that requires prophylaxis and monitoring for TLS. However, it is logistically easier than venetoclax plus obinutuzumab. The most common side effects are low white blood cell count, diarrhea, and high blood pressure.

There is no single "best" standard treatment regimen for symptomatic CLL. Your doctor will work with you to recommend an approach based on your situation (including your age, general health, and whether you have other medical problems) as well as your preferences. He or she will also factor in your cancer's stage and whether it has certain genetic characteristics that influence which treatment options are available. Because this area is still evolving and different regimens are being studied, all people with CLL are advised to enroll in a clinical trial, if possible. (See 'Clinical trials' below.)

While some people who undergo treatment for CLL do achieve "complete remission" (meaning blood counts have returned to normal and there are no signs or symptoms of disease), the reality is that nearly everyone will experience a relapse eventually. (See 'Treatment of relapsed or refractory chronic lymphocytic leukemia' below.)

TREATMENT OF RELAPSED OR REFRACTORY CHRONIC LYMPHOCYTIC LEUKEMIA — Most people with CLL respond to initial treatment, but then develop "relapsed" disease at some point after treatment ends. A small number of people do not respond to initial treatment at all; this is called "refractory" disease.

Choosing treatment — Several different approaches can be used to treat relapsed or refractory CLL, depending on your situation, which regimen(s) you have had before, and how effective they were. As examples:

If you had a prolonged treatment-free period after with either venetoclax plus obinutuzumab or ibrutinib plus venetoclax, you might be a candidate for treatment with a venetoclax-based regimen. (See 'Advanced or symptomatic CLL' above.)

If you were treated initially with a covalent Bruton tyrosine kinase (BTK) inhibitor (acalabrutinib, zanubrutinib, or ibrutinib), the choice of subsequent therapy depends on the reason for discontinuation.

If you discontinued the BTK inhibitor due to side effects, you might be treated with a different BTK inhibitor. People with significant side effects with one BTK inhibitor may be able to tolerate a different one.

If the CLL progressed while you were taking the BTK inhibitor, it is unlikely to respond to the other covalent BTK inhibitors. Options include venetoclax as well as other targeted therapies, such as the noncovalent BTK inhibitor pirtobrutinib (brand name: Jaypirca), idelalisib (brand name: Zydelig), and duvelisib (brand name: Copiktra), some of which are given in combination with an antibody.

Depending on your individual situation, you can discuss the following options with your doctor:

Participation in a clinical trial – New drugs are in development for people with CLL that target cellular processes that have not been targeted before (see 'Clinical trials' below)

Stem cell transplantation (also called bone marrow transplantation or hematopoietic stem cell transplantation) (see 'Stem cell transplantation' below)

Treatment to reduce CLL-related symptoms and complications (see 'Treatment of chronic lymphocytic leukemia complications' below)

Stem cell transplantation — Stem cell transplantation (also called bone marrow transplantation or hematopoietic cell transplantation) is being more seriously considered as a therapy for people with high-risk CLL (eg, disease that is refractory to therapy or has certain genetic characteristics), especially for people under the age of 65 years. People who choose stem cell transplantation are usually given other therapies to control the CLL while preparing for the transplant with the goal of achieving a complete or partial remission.

Prior to a stem cell transplant, "conditioning" therapy is given with chemotherapy or radiation. This kills cancer cells but also destroys normal cells developing in the bone marrow. After conditioning, the person needs to have a healthy supply of very young blood cells, called stem cells, reintroduced or transplanted. The transplanted cells then re-establish the blood cell production process in the bone marrow. (See "Patient education: Hematopoietic cell transplantation (bone marrow transplantation) (Beyond the Basics)".)

The type of stem cell transplant used for people with CLL is called an "allogeneic" transplant. In allogeneic transplant, the person is given stem cells from a donor, ideally a brother or sister with a similar genetic make-up. If the person doesn't have a "matched" sibling, an unrelated person with a partially matched genetic makeup may be used.

Today, most CLL transplants are done as a reduced intensity transplant (called a "mini"-transplant or non-myeloablative transplant) from a relative or a matched unrelated donor and may achieve long-term control of their CLL.

TREATMENT OF CHRONIC LYMPHOCYTIC LEUKEMIA COMPLICATIONS — The major complications of CLL are caused by the low blood counts and immune system problems that arise either from the disease itself or the treatment.

Infection — Infection is one of the most serious risks associated with CLL treatment. Although less common, serious infections can also occur in people who have not yet received any treatment for their CLL. The most common infections affect the upper respiratory tract (eg, the sinuses and bronchi). Smokers are especially vulnerable, so if you smoke, it's very important to try to quit as soon as possible. Your doctor or nurse can help you if you are having trouble quitting. (See "Patient education: Quitting smoking (Beyond the Basics)".)

To help prevent infections, it's very important to make sure your vaccinations are up-to-date, including getting the pneumococcal (pneumonia) vaccine, the influenza (flu) vaccine, and the COVID-19 vaccine. Your doctor may also talk with you about receiving the respiratory syncytial virus (RSV) vaccine and the recombinant (not "live") zoster vaccine. However, "live" vaccines (such as the measles, mumps, rubella vaccine) should be avoided, particularly if you are undergoing CLL treatment. Talk to your doctor about which vaccines you need and whether any should be avoided. It's also important that your family members get the yearly flu vaccine to help protect you as well as themselves.

If you develop a respiratory tract infection, you may need treatment with antibiotics. Infection may be related to low levels of infection-fighting proteins called immunoglobulins. For this reason, people who have repeated infections and low immunoglobulin levels may be treated with intravenous immune globulin (also called IVIG or IGIV) to increase their immunoglobulin levels and lower the chance of infection. IVIG treatment can decrease the incidence of minor infections but may not decrease the incidence of serious infections.

Certain treatments used in CLL make you more susceptible to infections. In some cases, medications that promote the growth of new blood cells may be given to boost the white cell count and decrease the infection risk. People receiving certain drugs may need to take preventive antibiotics and may need to have special testing to monitor for infections.

Anemia — Anemia, or low red blood cell counts, is common in CLL. Red blood cells are needed to carry oxygen to all the cells in the body. People with anemia may experience fatigue, weakness, and chest pain. A blood transfusion may be needed to treat severe anemia in certain cases.

Low platelet counts — Platelets are important components of the blood's clotting mechanism. Without adequate numbers of platelets, internal and external bleeding can occur. People with CLL and low platelet counts may see their counts improve with treatment of the CLL. In some cases, platelet transfusions are needed. In some people with CLL, the immune system destroys healthy platelets (this is called immune thrombocytopenia). In this situation, platelet counts may improve after the use of steroids or other treatments that suppress the body's immune response.

Psychological aspects — People with CLL must live with the uncertainty associated with having a chronic serious illness. It can be frightening to learn that you have leukemia, and it might be scary or confusing if your doctor recommends not getting treatment right away. It helps to develop a strong relationship with your doctors and nurses and to feel comfortable speaking openly and honestly and asking questions. Some people and their families also find that counseling or participation in support groups can help them to cope with the strong emotions that can accompany a cancer diagnosis.

CLINICAL TRIALS — Many people with leukemia will be asked about enrolling in a clinical (research) trial. A clinical trial is a carefully controlled way to study the effectiveness of new treatments or new combinations of known therapies. Ask your doctor for more information, or read about clinical trials at:

https://www.cancer.gov/research/participate/clinical-trials

https://clinicaltrials.gov/

Videos addressing common questions about clinical trials are available from the American Society of Clinical Oncology (www.cancer.net/research-and-advocacy/clinical-trials/welcome-pre-act).

WHERE TO GET MORE INFORMATION — Your health care provider is the best source of information for questions and concerns related to your medical problem.

This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for health care professionals, are also available. Some of the most relevant are listed below.

Patient level information — UpToDate offers two types of patient education materials.

The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.

Patient education: Leukemia in adults (The Basics)
Patient education: Chronic lymphocytic leukemia (CLL) (The Basics)
Patient education: Neutropenia and fever in people being treated for cancer (The Basics)

Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.

Patient education: Hematopoietic cell transplantation (bone marrow transplantation) (Beyond the Basics)

Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.

Classification of hematopoietic neoplasms
Evaluating response to treatment of chronic lymphocytic leukemia
Hematopoietic cell transplantation in chronic lymphocytic leukemia
Overview of the treatment of chronic lymphocytic leukemia
Risk of infections in patients with chronic lymphocytic leukemia
Prevention of infections in patients with chronic lymphocytic leukemia
Overview of the complications of chronic lymphocytic leukemia
Clinical features and diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma
Pathobiology of chronic lymphocytic leukemia
Staging and prognosis of chronic lymphocytic leukemia
Treatment of relapsed or refractory chronic lymphocytic leukemia

The following organizations also provide reliable health information.

National Library of Medicine

(https://medlineplus.gov/healthtopics.html)

National Cancer Institute

(www.cancer.gov/types/leukemia/patient/cll-treatment-pdq)

American Cancer Society

(www.cancer.org)

The Leukemia & Lymphoma Society

(www.lls.org)

The Lymphoma Research Foundation

(https://lymphoma.org/)

National Marrow Donor Program

(bethematch.org/)

The American Society of Hematology

(www.hematology.org)

The American Society of Clinical Oncology

(www.cancer.net/cancer-types/leukemia-chronic-lymphocytic-cll)

The Alliance for Clinical Trials in Oncology

(www.allianceforclinicaltrialsinoncology.org)

Deutsche CLL Studengruppe

(http://www.dcllsg.de/)

ACKNOWLEDGMENT — UpToDate would like to acknowledge Michael J Keating, MD, who contributed to earlier versions of this topic review.

Disclaimer: This generalized information is a limited summary of diagnosis, treatment, and/or medication information. It is not meant to be comprehensive and should be used as a tool to help the user understand and/or assess potential diagnostic and treatment options. It does NOT include all information about conditions, treatments, medications, side effects, or risks that may apply to a specific patient. It is not intended to be medical advice or a substitute for the medical advice, diagnosis, or treatment of a health care provider based on the health care provider's examination and assessment of a patient's specific and unique circumstances. Patients must speak with a health care provider for complete information about their health, medical questions, and treatment options, including any risks or benefits regarding use of medications. This information does not endorse any treatments or medications as safe, effective, or approved for treating a specific patient. UpToDate, Inc. and its affiliates disclaim any warranty or liability relating to this information or the use thereof. The use of this information is governed by the Terms of Use, available at https://www.wolterskluwer.com/en/know/clinical-effectiveness-terms. 2024© UpToDate, Inc. and its affiliates and/or licensors. All rights reserved.
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