Chronic lymphocytic leukemia (CLL)

Causes

Chronic lymphocytic leukemia (CLL) causes a slow increase in white blood cells called B lymphocytes, or B cells. Cancer cells spread through the blood and bone marrow, 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

Exams 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
• Flow cytometry test of the white blood cells

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

If you have early stage CLL, your doctor will closely monitor you. Usually, no medicines or other treatment is given for early-stage CLL, unless you have:

• A high-risk type of CLL
• Infections that keep coming back
• Leukemia that is rapidly getting worse
• Low blood and platelet counts
• Fatigue, loss of appetite, weight loss, or night sweats
• Painful swollen lymph nodes

Chemotherapy medicines are used to treat CLL. Common ones include:

• Fludarabine, chlorambucil, cyclophosphamide (Cytoxan), and rituximab (Rituxan) used alone or together.
• Alemtuzumab (Campath) for patients with CLL that did not get better with fludarabine.
• Bendamustine for 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.

Outlook (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.

Possible 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

When to Contact a Medical Professional

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

Alternative Names

CLL; Leukemia - chronic lymphocytic (CLL)

References

Kantarjian H, O'Brien S. The chronic leukemias. In: Goldman L, Ausiello D, eds. Cecil Textbook of Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011: chap 190.

National Comprehensive Cancer Network. National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology: Non-Hodgkin’s Lymphomas. 2012. Version 1.2012.

Update Date: 3/14/2012

Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine. Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.