Leukemia - Chronic T-Cell Lymphocytic

Treatment Options

This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. 

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team.

The goal of treatment for a blood cancer is to bring about a remission (absence of cancer symptoms; also called “no evidence of disease” or NED). Descriptions of the most common treatment options for T-cell leukemia are listed below, followed by an outline of the treatment options by each subtype. Treatment options and recommendations depend on several factors, including the type and subtype of leukemia, possible side effects, and the patient’s preferences and overall health. 

Active surveillance/watch and wait

This approach means that the doctor closely monitors the patient, and active treatment begins only when the leukemia shows signs of worsening. It may also be called active surveillance or watchful waiting. Patients are monitored using blood and other tests at regularly scheduled checkups to track blood cell counts and look for other signs that the leukemia is worsening. Studies have shown that, for people with certain disease characteristics, the watch-and-wait approach is not harmful when compared with early treatment. Treatment begins when people develop signs that the disease is worsening, such as increasing fatigue, night sweats, enlarged lymph nodes, or falling blood cell counts. People with leukemia are encouraged to talk with their doctors about whether their symptoms need treatment, and to consider the benefits of treatment compared with the side effects of treatment.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication, or a hematologist, a doctor who specializes in treating blood disorders. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. 

Immunotherapy

Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function. Recombinant interferon alpha (Alferon, Roferon-A, Intron A) is used as a treatment for ATLL. Interferon is a natural protein found in the body that stimulates the immune system. 

Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells.

Recent studies show that not all cancers have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors involved in your leukemia. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them. 

For T-cell leukemia, a type of targeted therapy called monoclonal antibodies may be used. In particular, monoclonal antibodies are targeted against specific proteins on the surface of the leukemia cells, which can kill some of the leukemia cells. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Radiation therapy

Radiation therapy is the use of x-rays or other high-energy particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Side effects from radiation therapy include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.  

Surgery

Surgery to remove the spleen (splenectomy), which also makes white blood cells, may be recommended for some patients. A surgical oncologist is a doctor who specializes in treating cancer using surgery. 

Stem cell transplantation/bone marrow transplantation

A stem cell transplant is a medical procedure in which diseased bone marrow is replaced by highly specialized cells, called hematopoietic stem cells. Hematopoietic stem cells are found both in the bloodstream and in the bone marrow. Today, this procedure is more commonly called a stem cell transplant, rather than bone marrow transplant, because it is the blood stem cells that are typically being transplanted, not the actual bone marrow tissue.

Stem cell transplantation is not a common treatment option for people with T-cell leukemia, because it is not always an effective treatment for this disease and because many patients with this disease are older and the risks of the procedure are higher.  Before recommending transplantation, doctors will talk with the patient about the risks of this treatment and consider several other factors, such as the type of cancer, results of any previous treatment, and patient’s age and general health.

There are two types of stem cell transplantation depending on the source of the replacement blood stem cells: allogeneic (ALLO) and autologous (AUTO). AUTO transplants are not often used for patients with chronic T-cell leukemias.

In both types, the goal of transplantation is to destroy disease cells in the marrow, blood, and other parts of the body and allow replacement blood stem cells to create healthy bone marrow. In most stem cell transplants, the patient is treated with high doses of chemotherapy and/or radiation therapy to destroy as many cancer cells as possible. 

Palliative/supportive care

Leukemia and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the disease, an important part of care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the leukemia and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem, so it is addressed as quickly as possible. 

Treatments specific to T-cell leukemia type

LGLL. LGLL sometimes needs no treatment other than the watch-and-wait approach during its early stages, but treatment may begin once symptoms develop. Sometimes, low levels of neutrophils cause infections that need antibiotic treatment. When treatment for the disease is needed, LGLL can be treated with drugs that lower the immune system, such as cyclosporine (multiple brand names), cyclophosphamide (Cytoxan, Neosar), or low-dose methotrexate (multiple brand names). Treatment with oral cyclosporine may be recommended when low levels of neutrophils or platelets cause a major problem. Treatment with growth factors, such as filgrastim (Neupogen), which can stimulate the growth of neutrophils is sometimes used when infections because of low neutrophil counts become a problem. Treatment with combination chemotherapy is sometimes used if the disease is worsening quickly. Combination chemotherapy is similar to that used for aggressive lymphoma. 

T-PLL. T-PLL may be treated with drugs that include fludarabine (Fludara), chlorambucil (multiple brand names), cyclophosphamide, doxorubicin (Adriamycin), vincristine (Oncovin, Vincasar), pentostatin (Nipent), and prednisone (multiple brand names). Alemtuzumab (Campath) is a monoclonal antibody (see Targeted therapy above) that targets a specific protein on the surface of certain T cells and has helped manage T-PLL for some patients. 

Adult T-cell leukemia/lymphoma (ATLL). ATLL may be treated with zidovudine (Retrovir) and recombinant interferon alpha if it is in the chronic or acute phase. The goal of treatment is to strengthen the immune system and treat the human T-cell leukemia virus (HTLV). The lymphoma phase is usually treated with combination chemotherapy.

Sezary syndrome. Treatments for Sezary syndrome may be focused on the skin and include systemic whole-body treatments. Skin treatments include skin creams; phototherapy (the use of light to kill cancer cells); and radiation therapy, including total skin electron-beam radiation therapy, which can treat the entire surface of the skin. Systemic treatments for Sezary syndrome include chemotherapy, oral bexarotene (Targretin; a drug that is similar to vitamin A), denileukin diftitox (Ontak), an antibody which helps deliver a drug directly to the leukemia cells, alpha interferon, and sometimes ALLO stem cell transplantation. Alemtuzumab may also be effective for treating this disease. Recently, vorinostat (Zolinza) and romidepsin (Istodax) were approved by the U.S. Food and Drug Administration (FDA) for cutaneous T-cell lymphoma after other treatments do not work. The choice of treatment depends on the extent of the disease, as well as other factors.

Recurrent T-cell leukemia

A remission is when leukemia cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED. 

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the leukemia will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the leukemia returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the disease does return. 

If the leukemia does return after the original treatment, it is called recurrent leukemia. When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as chemotherapy, immunotherapy, targeted therapy, and stem cell transplantation) but may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent leukemia.

People with recurrent leukemia often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. 

Refractory T-cell leukemia

If the leukemia continues to worsen despite treatment, it is called refractory leukemia.

Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this type of leukemia, because there can be different opinions about the best treatment plan or whether further treatment is recommended. 

Your health care team may recommend a treatment plan that includes a combination of the treatments discussed above (chemotherapy, immunotherapy, targeted therapy). Supportive care will also be important to help relieve symptoms and side effects.

For many patients, a diagnosis of refractory leukemia can be very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

If treatment fails

Recovery from leukemia is not always possible. If treatment is not successful, the disease may be called advanced or terminal leukemia.

This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Palliative care given toward the end of a person’s life is called hospice care. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. 

Side Effects