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) that begins in the stem cells of the bone marrow and then invades the blood. Overtime, CLL may also spread to the lymph nodes and other organs including the liver, spleen and lungs.^[1] It occurs when the stem cells that make lymphocytes become out of control and produce increasing amounts of abnormal lymphocytes (also called leukemic cells). Eventually, these abnormal cells replace normal lymphocytes and can crowd out other types of normal blood cells, leading to the features of the condition.^[1] Early signs and symptoms of CLL may include fatigue, weight loss, loss of appetite, fever, night sweats and/or frequent infections.^[2]^[3] The exact underlying cause of CLL is unknown; however, approximately 5% of affected people have other family members with the condition, which suggests there may be a genetic component in rare cases. The best treatment depends on many factors including the stage of the condition, the age of the affected person, the blood cell counts, whether the CLL has recurred, and the signs and symptoms present in each person.^[4]^[1]

Last updated: 12/27/2014

Symptoms Symptoms

Chronic lymphocytic leukemia (CLL) is the most common form of leukemia in adults and is rarely diagnosed in people under age 45.^[2] Many people affected by CLL have no symptoms at the time of diagnosis. In these cases, the condition is often identified incidentally (by chance) during a routine blood test. The symptoms of CLL usually develop slowly although the condition can progress more quickly in rare cases.^[1]

The signs and symptoms of CLL vary, but may include:^[2]^[1]^[4]^[3]

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

Last updated: 12/28/2014

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Autosomal dominant somatic cell mutation	-
Cellular immunodeficiency	-
Chronic lymphatic leukemia	-
Somatic mutation	-

Last updated: 9/1/2017

Cause Cause

The exact underlying cause of chronic lymphocytic leukemia (CLL) is unknown. Although approximately 5% of people affected by CLL have other family members with the condition, no genetic cause has been identified.^[5]

Factors that increase the risk for CLL include:^[1]^[4]^[2]

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 (a pesticide used to kill unwanted plants) during the Vietnam War

It is important to note that although these factors increase the risk for CLL, it does not mean that CLL will develop; not having one of these risk factor does not mean that CLL will not develop.

Last updated: 12/28/2014

Inheritance Inheritance

In most cases, chronic lymphocytic leukemia (CLL) is considered an acquired (non-inherited) condition. Approximately 5% of people affected by CLL have other family members with the condition, which suggests that there may be a genetic component in rare cases. However, scientists have yet to identify a disease-causing gene or combination of genes that is associated with CLL.^[5]

Last updated: 12/28/2014

Diagnosis Diagnosis

Chronic lymphocytic leukemia (CLL) is often discovered incidentally (by chance) when abnormally high levels of white blood cells are observed during a routine blood test. A diagnosis of CLL is usually confirmed by a bone marrow biopsy, a lymph node biopsy and/or blood tests (i.e. complete blood count, flow cytometry). A complete blood count measures the number of each type of blood cell, while flow cytometry closely examines blood cells to determine if they are cancerous.^[1]^[4]^[3]

Other specialized laboratory testing may be ordered to gain information regarding the course of CLL. These tests may help predict the response to treatment and/or the likelihood of relapse.^[4]^[3]

For more information about the tests used to diagnose or predict the prognosis (long-term outlook) of CLL, please click here.

Last updated: 12/28/2014

Treatment Treatment

The best treatment for chronic lymphocytic leukemia (CLL) depends on many factors, including the stage of the condition, the age of the affected person, the blood cell counts, whether the CLL has recurred, and the signs and symptoms present in each person. For example, people affected by an early stage of CLL who have not yet developed any signs or symptoms of the condition may simply be closely monitored by their physician without any treatment (watchful waiting). Once the condition progresses and symptoms develop, treatments may include chemotherapy, radiation therapy, surgery, and/or targeted therapies (a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells).^[1]^[4] Bone marrow or stem cell transplantation may be used in younger patients with advanced or high-risk CLL.^[2]

For more information about the treatment of CLL, please click here.

Last updated: 12/28/2014

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
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
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: Treatment of patients with chronic lymphocytic leukemia (CLL) with 17p deletion, as detected by an FDA approved test, who have received at least one prior therapy.
National Library of Medicine Drug Information Portal
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 long-term outlook (prognosis) for people with chronic lymphocytic leukemia (CLL) varies based on many factors, including the stage of the condition; whether abnormal lymphocytes have spread throughout the bone marrow and to other parts of the body; the affected person's response to treatment; and the affected person's general health.^[1] Approximately half of people diagnosed in the early stages of CLL live more than 12 years.^[2] Later stages of CLL are often associated with significant health problems and disability, both from the condition and from complications of treatment.^[4]

Last updated: 12/28/2014

Do you have updated information on this condition? Let us know.

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 studying or have studied 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

Focus On Chronic Lymphocytic Leukemia
115 Broadway, Suite 1301
New York, NY 10006
Telephone: 212-349-2910
E-mail: LRF@lymphoma.org
Website: http://www.focusoncll.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: 800-955-4572
Telephone: 914-949-5213
Fax: 914-949-6691
E-mail: infocenter@lls.org
Website: http://www.lls.org/

Do you know of an organization? Send us your suggestions.

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.

Genetics 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 GeneTests, 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.

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.

News & Events News & Events

News

Don’t Miss the NCATS Toolkit for Patient-Focused Therapy Development Event
August 31, 2017
Funding Opportunities Now Available for the Undiagnosed Diseases Network (UDN) Phase II
August 30, 2017
IRDiRC Goals 2017-2027: New Rare Disease Research Goals for the Next Decade
August 10, 2017

NCATS Co-Sponsored Conferences

Advancing Symptom Clusters Research on Rare Cancers Wednesday, June 17, 2015
Location: NIH Neuroscience Building, Bethesda, MD
Description:
It is intended that the in-depth, interdisciplinary dialogue of this expert working group will formulate an emerging consensus on a working definition of symptom clusters and, specific gaps and opportunities that provide a foundation for a transformative strategic blueprint to guide future symptom cluster research in rare cancers. In addition, it is anticipated that workshop proceedings will inform a funding opportunity announcement.

Contact: Sue Marden, Ph.D.,(301) 496-9623,mardens@mail.nih.gov

Co-funding Institute(s): National Institute of Nursing Research, Office of Rare Diseases Research
Overcoming Barriers to International Clinical Trials for Rare Cancers Friday, December 10, 2010
Location: Bethesda, Maryland
Description: The goals of this conference were to introduce key institutional players to the topic of international clinical trials in rare cancers and to establish an ongoing dialogue. Participants left the meeting with a set of specific priorities that need to be enacted to promote these trials. The meeting promoted consensus on the way that resources are prioritized to address rare cancers. Participants were asked to convey the content of the meeting to their constituencies and to follow up with pilot concepts.

Contact: Jack Welch, M.D., Ph.D., jack.welch@nih.govjack.welch@nih.gov

Co-funding Institute(s): National Cancer Institute, Office of Rare Diseases Research
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