Chronic lymphocytic leukemia

Title

Other Names:

Leukemia, lymphocytic, chronic; CLL

Categories:

Rare Cancers

Summary Summary

Chronic lymphocytic leukemia (CLL) is a cancer of the lymphocytes (a type of white blood cell). It begins in the stem cells of the bone marrow and then spreads to the blood. Over time, CLL may also spread to the lymph nodes and other organs, including the liver, spleen and lungs.^[1] Early signs and symptoms of CLL may include swollen lymph nodes, fatigue, weight loss, loss of appetite, fever, night sweats and/or frequent infections.^[1]^[2] The underlying cause of CLL is unknown. It usually occurs in people with no family history of the condition, but familial cases have been reported.^[3]^[4] Treatment options depend on many factors, including the stage of the condition, blood cell counts, and whether the CLL has recurred (come back).^[1]^[3] Treatment may not be necessary until a person develops symptoms or there is other evidence of rapid cancer progression.^[2]^[3] The chance of recovery and life expectancy vary from person to person. While most people with CLL live at least 5 to 10 years, some do not survive more than 2 to 3 years after the diagnosis.^[3]

Last updated: 10/9/2017

Symptoms Symptoms

Many people with CLL have no symptoms at the time of diagnosis. In these cases, the condition is often found incidentally (by chance) during a routine blood test. Symptoms usually develop slowly, although CLL can progress quickly in rare cases.^[1]

The signs and symptoms of CLL vary, but may include:^[1]^[2]^[3]

Enlarged lymph nodes
Enlarged liver and/or spleen
Night sweats
Fever
Fatigue
Loss of appetite
Weight loss
Frequent infections
Abnormal bruising

Last updated: 10/9/2017

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

Showing of 4 |

Medical Terms	Other Names	Learn More: HPO ID
Percent of people who have these symptoms is not available through HPO
Autosomal dominant somatic cell mutation		0001444
Cellular immunodeficiency		0005374
Chronic lymphatic leukemia		0005550
Somatic mutation		0001428

Showing of 4 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 11/1/2019

Do you have updated information on this disease? We want to hear from you.

Cause Cause

The underlying cause of chronic lymphocytic leukemia (CLL) is not known.^[3]^[4] It is believed that CLL occurs following a pre-cancerous condition known as monoclonal B-cell lymphocytosis (MBL). However, MBL is present in 5 to 15 percent of people over the age of 60. It is not known why a very small percentage of people with MBL develop CLL.^[5]

While familial cases have been reported, no genes directly responsible for CLL have been identified.^[4] Scientists are studying specific regions of chromosomes to see if some people may inherit a genetic susceptibility to developing CLL.^[4]

Some factors appear to increase a person's risk for CLL. It is important to note that having one or more risk factors does not mean that a person will definitely develop CLL. Most people with risk factors will not develop CLL. Risk factors may include:^[1]^[2]^[3]

Being over age 45
Being male
Being white
Having other family members with CLL or other cancers of the lymph system
Having family members who are Russian Jews or Eastern European Jews
Being exposed to Agent Orange (an herbicide used to kill unwanted plants) during the Vietnam War

Last updated: 10/9/2017

Inheritance Inheritance

Chronic lymphocytic leukemia (CLL) is generally considered an acquired condition and usually occurs in people with no family history of CLL. However, familial cases have been reported.^[6] CLL and other cancers affecting the lymph system and blood occur more frequently in first-degree relatives of people with CLL than in the general population. There is currently no proof that CLL itself is inherited, but certain genes (or combinations of genes) may predispose a person to familial cancer, including CLL. A number different chromosomal regions are being studied to identify possible susceptibility genes for familial CLL.^[4] Having a genetic predisposition or susceptibility does not mean that a person will definitely develop CLL or another type of cancer; it means a person has an increased risk to develop cancer.

While cancer cells in CLL are found to have genetic changes or mutations, these changes are acquired (not inherited) and are limited to the cancer cells.^[6] They are called somatic mutations and are not passed on from parent to child.

Last updated: 10/11/2017

Diagnosis Diagnosis

Chronic lymphocytic leukemia (CLL) is often first suspected incidentally (by chance) during a routine blood test.^[1] In some cases, signs and symptoms suggest that a person might have CLL, but tests are needed to confirm the diagnosis.^[7] The tests that are needed may differ from person to person.

Blood tests that may be used to confirm a diagnosis of CLL include:^[7]

Complete blood count - people with CLL have too many lymphocytes (called lymphocytosis).
Peripheral blood smear - when blood is looked at under the microscope, it might show abnormal-looking lymphocytes.
Flow cytometry - looks for the characteristics of CLL cells, and may be used to determine the stage of the cancer.

While blood tests are often enough to diagnose CLL, other types of tests may be used for further confirmation, to see how advanced the cancer is, and/or predict a person's prognosis (outlook). These may include:^[3]^[7]

Bone marrow aspiration and biopsy - samples of bone marrow may be taken from the back of the hip bone or other bones. The samples are looked at under the microscope and may undergo various tests.
Lymph node biopsy - may be recommended if a lymph node has grown and there is concern the leukemia has developed into a more aggressive cancer.
Imaging tests (ultrasound, CT scan, or MRI) - these may be done if there is concern the cancer has spread to another organ.
Gene tests - cells from blood or bone marrow samples are grown in the lab so their chromosomes can be examined. Some cases of CLL have chromosome changes that can be seen under the microscope.

Last updated: 10/11/2017

Treatment Treatment

The best treatment for each person with chronic lymphocytic leukemia (CLL) depends on many factors, including whether a person has symptoms, the stage of the condition, results of various tests, and whether the CLL has recurred (come back).^[1] For example, people with early stage CLL who have no symptoms may simply be closely monitored by their doctor without any treatment (watchful waiting).^[2] When a person develops symptoms, or there are signs the cancer has progressed, treatment options may include:^[1]^[2]^[3]

Chemotherapy
Radiation therapy
Targeted therapy - drugs or other substances used to find and kill cancer cells without hurting normal cells. Examples include monoclonal antibody therapy, tyrosine kinase inhibitor therapy, and BCL2 inhibitor therapy.
Blood or platelet transfusions if blood counts are low
Bone marrow or stem cell transplantation - in younger patients with advanced or aggressive CLL
Surgery - for example, spleen removal (splenectomy) for enlarged spleen and low blood counts

More information about the treatment of CLL can be viewed on the National Cancer Institute's page about CLL treatment.

Last updated: 10/11/2017

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Ofatumumab (Brand name: Arzerra) - Manufactured by Novartis Pharmaceuticals Corporation
FDA-approved indication: Arzerra in combination with chlorambucil, for the treatment of previously untreated patients with chronic lymphocytic leukemia (CLL) for whom fludarabine-based therapy is considered inappropriate. Also Arzerra in combination with fludarabine and cyclophosphamide for the treatment of patients with relapsed chronic lymphocytic leukemia (CLL).
National Library of Medicine Drug Information Portal
Medline Plus Health Information
Alemtuzumab (Brand name: Campath) - Manufactured by Genzyme Corporation
FDA-approved indication: The treatment of patients with B-cell chronic lymphocytic leukemia who have been treated with alkylating agents and who have failed fludarabine therapy.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
PI3K-delta and PI3K-gamma inhibitor (Brand name: Duvelisib) - Manufactured by Verastem, Inc.
FDA-approved indication: September 2018, PI3K-delta and PI3K-gamma inhibitor (Duvelisib) was approved for the treatment of adult patients with relapsed or refractory chronic lymphocytic leukemia (CLL), small lymphocytic lymphoma (SLL), or follicular lymphoma after at least two prior therapies.
National Library of Medicine Drug Information Portal
Buffered intrathecal electrolyte/dextrose injection (Brand name: Elliotts B Solution) - Manufactured by QOL Medical, LLC
FDA-approved indication: For the intrathecal administration of methotrexate and cytarabine for the prevention or treatment of meningeal leukemia and lymphocytic lymphoma.
National Library of Medicine Drug Information Portal
Fludarabine phosphate (Brand name: Fludara) - Manufactured by Berlex Laboratories, Inc.
FDA-approved indication: Treatment of chronic lymphocytic leukemia (CLL), including refractory CLL.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
Fludarabine phosphate oral tablets (Brand name: Fludarabine (oral)) - Manufactured by Antisoma
FDA-approved indication: Treatment of adult patients with B-cell chronic lymphocytic leukemia (CLL) whose disease has not responded to or has progressed during or after treatment with at least one standard alkylating-agent containing regimen.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
Obinutuzumab (Brand name: Gazyva) - Manufactured by Genentech, Inc.
FDA-approved indication: Treatment of patients with previously untreated chronic lymphocytic leukemia in combination with chlorambucil.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
Ibrutinib (Brand name: Imbruvica) - Manufactured by Pharmacyclics, Inc.
FDA-approved indication: Treatment of patients with chronic lymphocytic leukemia (CLL) who have received at least one prior therapy. Treatment of patients with chronic lymphocytic leukemia with or without 17p deletion.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
Rituximab and hyaluronidase human (Brand name: Rituxan Hycela) - Manufactured by Genentech, Inc.
FDA-approved indication: Treatment of adult patients with previously untreated and previously treated CLL in combination with fludarabine.
National Library of Medicine Drug Information Portal
Bendamustine hydrochloride (Brand name: Treanda) - Manufactured by Cephalon, Inc.
FDA-approved indication: Treatment of patients with chronic lymphocytic leukemia.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
Venetoclax (Brand name: Venclexta) - Manufactured by AbbVie Inc.
FDA-approved indication: June 2018, venetoclax (Venclexta) received expanded approval for the treatment of patients with chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL), with or without 17p deletion, who have received at least one prior therapy.
National Library of Medicine Drug Information Portal
Medline Plus Health Information
idelalisib (Brand name: Zydelig) - Manufactured by Gilead Sciences, Inc
FDA-approved indication: (1) Relapsed chronic lymphocytic leukemia (CLL), in combination with rituximab, in patients for whom rituximab alone would be considered appropriate therapy due to other co-morbidities; and (2) Relapsed small lymphocytic lymphoma (SLL) in patients who have received at least two prior systemic therapies; and (3) Relapsed follicular B-cell non-Hodgkin lymphoma (FL) in patients who have received at least two prior systemic therapies.
National Library of Medicine Drug Information Portal

Prognosis Prognosis

The chance of recovery and life expectancy for each person depends on many factors. These include:^[1]^[7]

The presence and type of genetic changes in CLL cells - for example, the loss of part of chromosome 13 is often associated with less aggressive disease and a better outlook, while changes in chromosomes 11 or 17 are linked to a poorer outlook.
Whether lymphocytes are spread throughout the bone marrow - small groups of cells are associated with a better outlook than if cells are scattered throughout the marrow.
The stage of the disease.
Whether the CLL is improving with treatment or has recurred (come back).
Whether the CLL develops into lymphoma or prolymphocytic leukemia.
The person's general health.

Most people diagnosed with CLL live 5 to 10 years. Often there are few health issues for several years, followed by 1 to 2 years of progressive disease. During this time, quality of life is diminished due to both the condition and complications of treatment.^[3] About half of people diagnosed in the early stages live more than 12 years.^[2] Lastly, some people do not survive more than 2 to 3 years after the diagnosis due to complications from CLL.^[3]

Last updated: 10/11/2017

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Chronic lymphocytic leukemia. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

CLL Society
PO Box 1390
Claremont, CA 91711
E-mail: https://cllsociety.org/contact-us/
Website: https://cllsociety.org/
Leukaemia Foundation
E-mail: info@leukaemia.org.au
Website: http://www.leukaemia.org.au/
Leukemia and Lymphoma Society
3 International Drive, Suite 200
Rye Brook, NY 10573
Toll-free: 1-(800) 955-4572 (patients and families)
Telephone: 1-(888) 557-7177 (general)
E-mail: https://www.lls.org/content/contact-us
Website: https://www.lls.org/
Lymphoma Research Foundation
Wall Street Plaza
88 Pine Street, Suite 2400
New York, NY 10005
Toll-free: 1-800-500-9976
Telephone: +1-212-349-2910
Fax: +1-212-349-2886
E-mail: helpline@lymphoma.org
Website: https://www.lymphoma.org/

Organizations Providing General Support

American Cancer Society
250 Williams Street NW
Atlanta, GA 30329
Toll-free: 1-800-227-2345
Website: https://www.cancer.org

Do you know of an organization? We want to hear from you.

Living With Living With

Living with a genetic or rare disease can impact the daily lives of patients and families. These resources can help families navigate various aspects of living with a rare disease.

Financial Resources

Patient Access Network Foundation (PAN Foundation) has Assistance Programs for those with health insurance who reside in the United States. The disease fund status can change over time, so you may need to check back if funds are not currently available.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The American Cancer Society provides information on Chronic lymphocytic leukemia. Please click on the link to access this resource.
The Leukemia and Lymphoma Society has an information page on Chronic lymphocytic leukemia. Click on the link to view this information page.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manual provides information on this condition for patients and caregivers.
The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Merck Manual for health care professionals provides information on Chronic lymphocytic leukemia.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
PubMed is a searchable database of medical literature and lists journal articles that discuss Chronic lymphocytic leukemia. Click on the link to view a sample search on this topic.

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