Many people with CLL & SLL do not require treatment right away and some people never require treatment. The decision to start treatment depends on whether you have signs or symptoms that are associated with progressive disease. Together, these signs and symptoms are called treatment indications. Treatment is started only if you have at least one of the treatment indications listed below:
- Evidence your bone marrow is unable to keep up with your body's need for healthy blood cells - measured by the development of, or worsening of, anemia (low red blood cell counts) and/or thrombocytopenia (low platelet counts);
- Massive spleen, progressive spleen enlargement, or an enlarged spleen that is causing symptoms;
- Massive lymph nodes (at least 10 cm in longest diameter), progressive lymph node enlargement or large lymph nodes that are causing symptoms;
- Increase in number of lymphocytes of more than 50% over a 2-month period or lymphocyte doubling time (LDT) of less than 6 months;
- Autoimmune anemia and/or thrombocytopenia (low numbers of red blood cells or platelets that results when your body makes antibodies that destroy them) that doesn’t respond to standard medications;
‘B’ symptoms, defined as any one or more of the following disease-related symptoms or signs:
• Unintentional weight loss of 10% or more within the previous 6 months;
• significant fatigue (inability to work or perform usual activities);
• fevers higher than 38.0°C for 2 or more weeks without other evidence of infection; or
• heavy night sweats for more than 1 month without evidence of infection.
Although no cure has been found yet for CLL & SLL, there are many treatment options that allow patients to feel well and live for years in good health.
The goals of treatment are to:
- Reduce the numbers of CLL cells in your blood, lymph nodes, spleen and bone marrow
- Relieve your symptoms and maximize your quality of life for as long as possible
The type of treatment you have will depend on a number of factors, including:
- Your age
- Your general health or fitness
- Any other illnesses you have
- What changes have happened to the genes and chromosomes of the CLL & SLL cells
Your doctors will take all these factors into account when planning your treatment.
Standard treatment options for CLL & SLL include:
- Watch & wait
- Drug therapy
- Biologic therapy
- Stem-cell transplantation
- Radiation therapy
Patients requiring treatment are often treated with drug therapy: chemotherapy and/or monoclonal antibody therapy. The choice of recommended treatment generally depends on the patient’s overall health status and the stage of his or her disease. Age may be a factor for certain types of therapy.
Fludarabine (Fludara®) is considered to be one of the most effective types of chemotherapy for CLL. Drug combinations, including fludarabine and cyclophosphamide (Cytoxan®)—FC; fludarabine and rituximab (Rituxan®)—FR; and fludarabine, cyclophosphamide and rituximab—FCR—are often used to treat patients with intermediate- and high-risk CLL. Studies comparing treatment with fludarabine alone to treatment with FC, or to treatment with various FCR regimens, have shown that combination treatments significantly improve the frequency of complete response in previously untreated people with CLL. Additional studies in clinical trials are needed to determine which patients will benefit from FCR versus other CLL treatments. In addition, treatments combining fludarabine with other drugs are currently being studied in clinical trials.
Bendamustine (Treanda®) is another type of chemotherapy that is approved for the treatment of CLL in some provinces. Combinations of bendamustine and other drugs, including rituximab and lenalidomide (Revlimid®), are currently being studied in clinical trials for the treatment of people with CLL.
Patients with CLL may receive chemotherapy as a single drug. Examples include:
- Chlorambucil may be administered orally with few immediate side effects
- Fludarabine is administered orally or intravenously and can be associated with some side effects.
Many patients with CLL will receive a combination chemotherapy regimen such as:
- FC chemotherapy (fludarabine and cyclophosphamide)
- CVP chemotherapy (cyclophosphamide, vincristine and prednisone)
- CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone).
Patients should be aware of all of their treatment options and speak to their doctors about selecting the best treatment for them.
Biologic therapy is a treatment that stimulates or restores the ability of your immune system to fight disease or infections.
Monoclonal antibodies are proteins that are bioengineered in the laboratory. Each monoclonal antibody therapy is designed to recognize a specific molecule on a cell. The monoclonal antibody therapy targets the molecule and attaches to the cell, causing the cell to die. Most chemotherapy affects normal tissue cells as well as CLL cells. Monoclonal antibody therapy may affect some normal lymphocytes but spare most other cells. Even though the infusion of a monoclonal antibody into a patient’s vein may cause a short period of fever, chills or low blood pressure, generally people experience less troubling side effects with monoclonal antibody therapy than with chemotherapy.
The monoclonal antibody therapies rituximab (Rituxan®) and alemtuzumab (Campath®) are used to treat people with CLL. Rituximab targets CD20 on the CLL cell’s surface; alemtuzumab targets CD52. Alemtuzumab is approved as a single agent for CLL treatment. Both rituximab and alemtuzumab have been very effective in treating many people with CLL.
These therapies continue to be studied in clinical trials. Other new monoclonal antibodies, such as ofatumumab (Arzerra®) and lumiliximab are also being studied in clinical trials, either alone or combined with chemotherapy, as treatment for previously untreated patients and for patients with refractory or relapsed CLL.
Patients with CLL may be candidates for stem-cell transplantation. In stem-cell transplantation, immature blood cells (stem cells) are taken from the blood or bone marrow of the patient (autologous transplantation) or a compatible donor (allogeneic transplantation). Stem-cell transplantation is an aggressive treatment that is still considered experimental but may be an option for younger CLL patients who no longer respond to treatment.
Radiation therapy may be used:
- To reduce the size of an enlarged spleen (rarely used)
- To reduce the size of a specific lymph node.
Surgery to remove an enlarged spleen is rarely necessary but can be done:
- To relieve the pressure caused by the enlarged spleen on other organs
- To stop the increased destruction of red blood cells and platelets.
Relapsed or Refractory CLL
“Relapsed CLL” is the term for disease that responded to therapy but, after six or more months, stopped responding. “Refractory disease” is the term for CLL that does not result in a remission (but may be stable) or disease that gets worse within six months of the last treatment.
People who are treated for relapsed or refractory CLL often have good quality years of remission after more treatment. Treatment guidelines for people with relapsed CLL are generally the same as treatment for newly diagnosed people.
People who have refractory CLL with a short time to progression after the first treatment and/or CLL cells with del 17p often do not respond to standard chemotherapy. These people are advised to speak to their physicians about whether or not treatment in a clinical trial is a good option for them. Investigative clinical protocols for drug therapies or allogeneic stem-cell transplantation may offer appropriate treatment options.