) become cancerous and gradually replace normal cells in lymph nodes.People may have no symptoms, or they may have general symptoms such as tiredness, fever, night sweats, and unintended weight loss.
People may also have enlarged lymph nodes and a sense of abdominal fullness due to enlargement of the spleen.
Blood tests are needed for diagnosis.
Treatment includes chemotherapy drugs, monoclonal antibodies, and sometimes radiation therapy.
(See also Overview of Leukemia Overview of Leukemia Leukemias are cancers of white blood cells or of cells that develop into white blood cells. White blood cells develop from stem cells in the bone marrow. Sometimes the development goes awry... read more )
More than three fourths of the people who have chronic lymphocytic leukemia (CLL) are older than 60, and the disease is extremely rare in children. CLL is the most common type of leukemia Overview of Leukemia Leukemias are cancers of white blood cells or of cells that develop into white blood cells. White blood cells develop from stem cells in the bone marrow. Sometimes the development goes awry... read more in North America and Europe. It is rare in Japan and Southeast Asia, which suggests that genetics plays some role in its development.
The number of cancerous, mature-appearing lymphocytes increases first in the blood, bone marrow, and lymph nodes. Cancerous lymphocytes then spread to the liver and spleen, both of which begin to enlarge.
In the bone marrow, cancerous lymphocytes may crowd out normal blood-producing cells, resulting in a decreased number of one or more of the following:
Red blood cells Red Blood Cells The main components of blood include Plasma Red blood cells White blood cells Platelets read more
, which carry oxygen in the bloodstreamWhite blood cells White Blood Cells The main components of blood include Plasma Red blood cells White blood cells Platelets read more
, which help defend the body against infection Platelets Platelets The main components of blood include Plasma Red blood cells White blood cells Platelets read more
, small cell-like particles that help in the clotting process
Cancerous lymphocytes do not function like normal lymphocytes and produce antibodies, proteins that help fight infections. The smaller number of healthy lymphocytes that remain are not always able to produce enough antibodies so infection is likely. Also, the immune system, which ordinarily defends the body against foreign organisms and substances, sometimes becomes misguided, reacting to and destroying normal tissues. This misguided immune activity can result in the destruction of red blood cells, neutrophils, or platelets.
CLL can sometimes transform into an aggressive cancer called lymphoma Overview of Lymphoma Lymphomas are cancers of lymphocytes, which reside in the lymphatic system and in blood-forming organs. Lymphomas are cancers of a specific type of white blood cells known as lymphocytes. These... read more 
. This type of transformation, called Richter's transformation, happens in 2 to 10 percent of cases.
Symptoms of CLL
In early stages of CLL, most people have no symptoms, and the disease is diagnosed only because of an increased white blood cell count. Later symptoms may include
Enlarged lymph nodes
Fatigue
Loss of appetite
Weight loss
Night sweats
Shortness of breath when exercising
A sense of abdominal fullness resulting from an enlarged spleen
As CLL progresses, people may appear pale and bruise easily. Bacterial, viral, and fungal infections may occur late in the course of the disease as the bone marrow produces fewer healthy white blood cells to fight infection.
Diagnosis of CLL
Blood tests
Sometimes CLL is discovered accidentally when blood counts ordered for some other reason show an increased number of lymphocytes. Specialized blood tests (called flow cytometry and immunophenotyping) to characterize the abnormal lymphocytes can be done on the cells in the blood. Blood tests also may show that the numbers of red blood cells, platelets, and antibodies are low.
A bone marrow evaluation Bone Marrow Examination Red blood cells, most white blood cells, and platelets are produced in the bone marrow, the soft fatty tissue inside bone cavities. Sometimes a sample of bone marrow must be examined to determine... read more is not necessary for the diagnosis of CLL but, if done, often shows an increased number of lymphocytes.
Prognosis for CLL
Usually CLL progresses slowly. Doctors determine how far the disease has progressed (staging) to predict the survival time. Staging is based on several factors, including the
Number of lymphocytes in the blood
Number of lymphocytes in the bone marrow
Size of the spleen
Size of the liver
Number of red blood cells in the blood
Number of platelets in the blood
People who are in the early stages of CLL often survive 10 to 20 years or longer after the diagnosis is made and usually do not need treatment in the early stages. People who have a low number of red blood cells (anemia Overview of Anemia Anemia is a condition in which the number of red blood cells is low. Red blood cells contain hemoglobin, a protein that enables them to carry oxygen from the lungs and deliver it to all parts... read more ) or a low number of platelets (thrombocytopenia Overview of Thrombocytopenia Thrombocytopenia is a low number of platelets (thrombocytes) in the blood, which increases the risk of bleeding. Thrombocytopenia occurs when the bone marrow makes too few platelets or when... read more 
) need more immediate treatment and have a less favorable prognosis. Usually, death occurs because the bone marrow can no longer produce a sufficient number of normal cells to carry oxygen, fight infections, and prevent bleeding.
For reasons probably related to changes in the immune system, people who have CLL are more likely to develop other cancers, such as skin cancers Overview of Skin Cancer Skin cancer is the most common type of cancer. Skin cancer is most common among people who work or play sports outside and among sunbathers. Fair-skinned people are particularly susceptible... read more or lung cancers Overview of Skin Cancer Skin cancer is the most common type of cancer. Skin cancer is most common among people who work or play sports outside and among sunbathers. Fair-skinned people are particularly susceptible... read more . CLL can also transform into a more aggressive type of cancer of the lymphatic system (lymphoma Overview of Lymphoma Lymphomas are cancers of lymphocytes, which reside in the lymphatic system and in blood-forming organs. Lymphomas are cancers of a specific type of white blood cells known as lymphocytes. These... read more ).
Treatment of CLL
Chemotherapy
Immunotherapy
Because CLL progresses slowly, many people do not need treatment for years, Doctors usually wait to begin treatment until one or more of the following occur
The number of lymphocytes begins to increase and cause symptoms
The lymph nodes begin to enlarge
The number of red blood cells or platelets decreases
Drug treatment of CLL
Drugs, including chemotherapy Chemotherapy and Other Systemic Cancer Treatments Systemic treatments are those that have effects throughout the body rather than being applied directly to the cancer. Chemotherapy is a form of systemic treatment that uses drugs to kill cancer... read more and immunotherapy Immunotherapy for Cancer Immunotherapy is used to stimulate the body's immune system against cancer. These treatments target specific genetic characteristics of the tumor cells. The genetic characteristics of tumors... read more (such as monoclonal antibodies), help relieve symptoms and shrink enlarged lymph nodes and spleen but do not cure the disease. Treatment can control CLL for many years and can often be used again with success when the leukemia regrows.
There is no standard combination of drugs that is used for CLL. For B-cell CLL, initial drug treatment usually includes drugs such as fludarabine and cyclophosphamide, which kill cancer cells by interacting with DNA. Currently, chemotherapy and a monoclonal antibody called rituximab are also used to treat CLL. This combination therapy usually is successful in controlling CLL (inducing remission). Eventually, most CLL becomes resistant to these drugs. Treatments with other drugs or other monoclonal antibodies are then considered.
Ibrutinib is a drug that has induced lasting remissions in some people with CLL. It can be used for initial treatment or for CLL that is unlikely to respond to other treatments, for CLL that fails to respond to other treatment (refractory) or for CLL that has relapsed. Obinutuzumab may be used in older people with both CLL and other illnesses. Sometimes, stem cell transplantation Stem Cell Transplantation Stem cell transplantation is the removal of stem cells (undifferentiated cells) from a healthy person and their injection into someone who has a serious blood disorder. (See also Overview of... read more can be done in people who have relapsed.
Treatment of CLL symptoms
Anemia is treated with blood transfusions Overview of Blood Transfusion A blood transfusion is the transfer of blood or a blood component from one healthy person (a donor) to a sick person (a recipient). Transfusions are given to increase the blood's ability to... read more 
and occasionally with injections of erythropoietin or darbepoietin (drugs that stimulate red blood cell formation). A low platelet count may cause bleeding and is treated with platelet transfusions. Infections are treated with antimicrobials. Radiation therapy is used to shrink enlarged lymph nodes or an enlarged liver or spleen if the enlargement is causing discomfort and chemotherapy is ineffective.
More Information
The following is an English-language resource that may be useful. Please note that the MANUAL is not responsible for the content of this resource.
Leukemia & Lymphoma Society: What is CLL: General information on many aspects of chronic lymphocytic leukemia, including diagnosis, treatment options, and research findings
Drugs Mentioned In This Article
| Generic Name | Select Brand Names |
|---|---|
fludarabine |
Fludara, Oforta |
cyclophosphamide |
Cyclophosphamide, Cytoxan, Neosar |
rituximab |
RIABNI, Rituxan, RUXIENCE, truxima |
ibrutinib |
Imbruvica |
obinutuzumab |
GAZYVA |
