Definition of Chronic lymphocytic leukemia (CLL):

Chronic lymphocytic leukemia is cancer of a type of white blood cells called lymphocytes.

See also:

• Acute lymphocytic leukemia (ALL)
• Chronic myelogenous leukemia (CML)
• Leukemia

Causes, incidence, and risk factors:

Chronic lymphocytic leukemia (CLL) causes a slow increase in white blood cells called B lymphocytes, or B cells. The cancerous cells spread from the blood marrow to the blood, and can also affect the lymph nodes or other organs such as the liver and spleen. CLL eventually causes the bone marrow to fail.

IThe cause of CLL is unknown. There is no link to radiation, cancer-causing chemicals, or viruses.

This cancer mostly affects adults, around age 70. It is rarely seen under age 40. The disease is more common in Jewish people of Russian or East European descent.

Symptoms:

Symptoms usually develop slowly over time. Many cases of CLL are detected by blood tests done in people for other reasons or who do not have any symptoms.

Symptoms that can occur include:

• Abnormal bruising (occurs late in the disease)
• Enlarged lymph nodes, liver, or spleen
• Excessive sweating, night sweats
• Fatigue
• Fever
• Infections that keep coming back (recur)
• Loss of appetite or becoming full too quickly (early satiety)
• Unintentional weight loss

Signs and tests:

Patients with CLL usually have a higher-than-normal white blood cell count.

Tests to diagnose and assess CLL include:

• Complete blood count (CBC) with white blood cell differential
• Bone marrow biopsy
• CT scan of the chest, abdomen, and pelvis
• Immunoglobulin testing
• Lactate dehydrogenase test

If your doctor discovers you have CLL, tests will be done to see how much the cancer has spread. This is called staging.

There are two systems used to stage CLL:

• The Rai system uses numbers 0 to IV to group CLL into low-, intermediate-, and high-risk categories. Generally, the higher the stage number, the more advanced the cancer.
• The Binet system uses letters A-C to stage CLL according to how many lymph node groups are involved and whether you have a drop in the number of red blood cells or platelets.

Some newer tests look at the chromosomes inside the cancer cells. The results can help your doctor better determine your treatment.

Treatment:

No treatment is usually give for early stage CLL. However, the patient must be closely monitored by their health care provider.

If chromosome testing suggests that you have a high-risk type of CLL, treatment may be started earlier.

Treatment may also be started if:

• Infections keep coming back
• Leukemia is growing rapidly.
• Low blood counts (anemia and thrombocytopenia (low platelet count) are present
• Fatigue, loss of appetite, weight loss, or night sweats occur

Several chemotherapy drugs are commonly used to treat CLL.

• Fludarabine, chlorambucil, cyclophosphamide (Cytoxan), and rituximab (Rituxan) may be used alone or in combination.
• Alemtuzumab (Campath) is approved for treatment of patients with CLL that have not responded to fludarabine.
• Bendamustine is a newer drug recently approved for use in patients with CLL that has come back after initial treatment.

Rarely, radiation may be used for painfully enlarged lymph nodes. Blood transfusions or platelet transfusions may be required if blood counts are low.

Bone marrow or stem cell transplantation may be used in younger patients with advanced or high-risk CLL. A transplant is the only therapy that offers a potential cure for CLL.

Expectations (prognosis):

How well a patient does depends on the stage of the cancer. About half of patients diagnosed in the early stages of the disease live more than 12 years.

Complications:

• Autoimmune hemolytic anemia
• Bleeding from low platelet count
• Hypogammaglobulinemia, a condition in which you have lower levels of antibodies, which increases your risk of infection
• Idiopathic thrombocytopenic purpura (ITP)
• Infections that keep coming back (recur)
• Overwhelming fatigue
• Other cancers, including a much more aggressive lymphoma (Richter' s transformation)
• Side effects of chemotherapy

Calling your health care provider:

Call health care provider if you develop enlarged lymph nodes or unexplained fatigue, bruising, excessive sweating, or weight loss.