Treatment options for people with chronic lymphocytic leukemia (CLL) vary greatly, depending on the disease stage and if the leukemia is causing any symptoms.

Low risk-CLL (Rai Stage 0): The prognosis for people with stage 0 CLL is very good with an average survival of nearly 15 years. Most of these patients are over age 60, and no curative treatment is currently available. The usual practice is to give no immediate treatment. Careful and frequent follow-up exams are recommended and treatment should be considered if there is evidence of progression of the leukemia or if the patient later develops bothersome symptoms. More than half of patients with low-risk CLL live at least 14 years after being diagnosed.

Intermediate-risk CLL (Rai Stage I and II): Patients with intermediate-risk CLL who do not have any symptoms may not need immediate treatment. Like those patients with low-risk CLL, they are followed later for signs of progression and onset of new symptoms. Half the people in this category live over 7 years.

The usual treatment for intermediate-risk CLL that is causing symptoms is chemotherapy with chlorambucil. Cyclophosphamide, may be substituted if chlorambucil causes side effects. Combinations of drugs have been also tried but have not been better than chlorambucil alone. Fludarabine is a chemotherapy drug that has been very useful in CLL. Oncologists usually reserve fludarabine for CLL that has recurred after treatment with other drugs. Recently, however, many are using fludarabine as the first treatment, particularly in younger people.

If enlargement of the spleen or lymph nodes in one region of the body is the only problem, localized treatment with low-dose radiation therapy is often used. Splenectomy (surgery to remove the spleen) is another option if enlargement of this organ causes symptoms. If very high numbers of leukemia cells are interfering with normal circulation, leukapheresis is used before chemotherapy. Leukapheresis involves passing the blood through a special machine that removes white blood cells (including leukemia cells) and returns the rest of the blood cells and plasma to the patient. The benefit of this treatment is immediate but temporary. Leukapheresis is useful because chemotherapy may not affect the number of cells until a few days after the first dose.

Clinical trials of interferons and other drugs that boost the immune system's response to the leukemia as well as trials of new chemotherapy drugs are an option for patients with intermediate-risk CLL.

High-risk CLL: As with intermediate-risk CLL, the usual treatment is chemotherapy with chlorambucil or cyclophosphamide, or fludarabine in younger patients, or when CLL persists (does not respond to treatment) or recurs after chlorambucil or cyclophosphamide treatment.

If enlargement of the spleen causes symptoms, surgical removal or radiation therapy (TBI) of the spleen may be helpful.

If several lymphoid tissue regions are affected, total body irradiation therapy is an option.

High-dose chemotherapy and total body irradiation with stem cell transplantation is being studied in clinical trials. Clinical trials of immunotherapy drugs, including interferon, and new chemotherapy drugs are currently being conducted.

The average survival time after diagnosis for patients with high-risk CLL is about 4 years.

Treatment of other complications of CLL: Sometimes CLL alters a patient's immune system in a way that causes it to attack his or her own red blood cells (immunohemolytic anemia) or blood platelets (immune-mediated thrombocytopenia). These conditions are treated with corticosteroid drugs such as prednisone, which is taken by mouth.

One of the most serious complications of CLL is transformation of the leukemia to a high-grade or aggressive non-Hodgkin's lymphoma. This is called Richter Syndrome or Richter Transformation. If this occurs, patients receive treatment for lymphoma. Refer to the American Cancer Society's document "Non-Hodgkin's Lymphoma (Adult)" for more information.

Patients with CLL rarely will have their leukemia transform into the acute form of lymphocytic leukemia. If this happens, then the patient will be treated with a chemotherapy regimen that is used on patients with acute lymphocytic leukemia. Refer to the American Cancer Society's document on "Leukemias-Adult Acute" for more information.