Treatment

The treatment of T-cell leukemia depends on the symptoms produced by the disease, whether the cancer has spread, and the person’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.

The goal of treatment for a blood cancer is to bring about a remission of the disease. A complete remission means the doctor can find no evidence of the disease.

This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials as a treatment option when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section.

General treatment options for T-cell leukemia are described below, followed by an outline of the treatment options by each subtype.

Active surveillance/watch and wait

This approach means that the doctor is closely monitoring the disease, and active treatment begins only when the leukemia shows signs of advancing. 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 symptoms that the leukemia is progressing. Studies have shown that, in people with certain disease characteristics, no harm comes from the watch-and-wait approach versus offering 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 versus side effects that might result.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. The side effects of chemotherapy depend on the individual and the dose used, but 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. Learn more about your prescriptions through Cancer.Net’s Drug Information Resources, which provides links to searchable drug databases.

Radiation therapy

Radiation therapy is the use of x-rays or other high-energy particles to kill cancer cells. To treat leukemia, radiation therapy usually comes from a machine outside the body and is called external-beam radiation therapy.

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 produces white blood cells, may occasionally be performed.

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 blood stem cells are typically what is being transplanted, not the actual bone marrow tissue.

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.

Stem cell transplantation is not a common treatment option for people with T-cell leukemia, because it is not a consistently effective therapy for this disease and because many patients with this disease are older and the risks of procedure are higher.

There are two types of stem cell transplantation depending on the source of the replacement blood stem cells: allogeneic (ALLO) and autologous (AUTO).

In an ALLO transplant, stem cells are obtained from a donor whose tissue matches the patient’s on a genetic level; this testing is called HLA-typing. Most often, a patient’s brother or sister serves as the donor, although unrelated donors can serve as the donor too. Millions of people worldwide have volunteered to donate stem cells for patients who do not have matched family members; matches can be made by searching a computer registry. In addition, a donation of stem cells derived from umbilical cord blood is sometimes considered if family donors are not available.

In an AUTO transplant, the patient’s own stem cells are used. The stem cells are obtained from the patient when he or she is in remission from previous treatment. The stem cells are then frozen until they are needed, usually after the high-dose treatment (explained below) is completed.

In both types, the goal of transplantation is to destroy cancer cells in the marrow, blood, and other parts of the body and have replacement blood stem cells 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. This also destroys the patient’s bone marrow tissue and suppresses the patient’s immune system so that, in an ALLO transplant, the donor cells are not rejected by the body. After the high-dose treatment is given, blood stem cells are infused into the patient’s vein to replace the bone marrow and restore normal blood counts from donor cells. Sometimes, ALLO transplants can also be performed after giving lower doses of chemotherapy and/or radiation therapy that are still sufficient to suppress the immune system and allow growth of the donor cells. (These transplants, sometimes termed “mini-transplants” or “reduced intensity transplants” have less immediate side effects, allowing the procedure to be used for older patients.)

For both ALLO and AUTO transplant types, the replacement cells engraft (begin to make new blood cells) and turn into healthy, blood-producing tissue in 10 days to three weeks. Destroying the patient’s own marrow reduces the body’s natural defenses, temporarily leaving the patient at an increased risk of infection. Until the patient’s immune system is back to normal, patients may need antibiotics and blood transfusions.

In an ALLO transplant, another major risk is that the donor’s cells will recognize the patient’s body as foreign, causing graft-versus-host disease (GVHD). GVHD may be a serious complication of allogeneic transplants and can be fatal. Other side effects may include liver problems, diarrhea, infections, and rashes. However, GVHD can also be a benefit, in that the donor cells can recognize the cancer cells as foreign and destroy these cells, a mechanism that is one of the major reasons why ALLO transplantation generally works so well over the long term. The risk of GVHD can be reduced with exact HLA-type matching and the use of preventative drugs.

In an AUTO transplant, there is little risk of GVHD because the replacement stem cells are the patient’s own cells. However, there is a risk in an autologous transplant that some of the cells that are put back into the patient could still be cancerous.

Learn more by reading the Cancer.Net Feature series Understanding Bone Marrow and Stem Cell Transplantation.

Immunotherapy

Immunotherapy or biologic therapy is designed to boost the body's natural defenses to fight the cancer. It uses materials either made by the body or in a laboratory to bolster, target, or restore immune system function. Of these, recombinant interferon alpha (Alferon N, Roferon-A, Intron A) has been found to be an effective therapy in ATLL. Interferon is a natural protein present in the body that stimulates immunity.

Monoclonal antibodies

Monoclonal antibodies are antibodies directed against specific proteins on the leukemic cell surface. When the antibody attaches to their antigen, some leukemic cells die.

Treatments specific to T-cell leukemia type

LGLL. LGLL sometimes requires no treatment beyond watch-and-wait during its early stages, but treatment may begin once symptoms develop. Sometimes, infections requiring treatment with antibiotics occur because of the limited numbers of neutrophils. When treatment for the disease does become necessary, LGLL is treated with immunosuppressant drugs, such as cyclosporine (multiple brand names), cyclophosphamide (Clafen, Cytoxan, Neosar), or low-dose methotrexate (multiple brand names). Treatment with oral cyclosporine, a drug that suppresses the immune system, can be effective in some people in whom low levels of neutrophils or platelets represent the major problem. Treatment with growth factors (granulocyte colony stimulating factors, Neupogen) which can stimulate the growth of neutrophils, are sometimes used when infections, due to low neutrophil counts, become a problem. Treatment with combination chemotherapy (the use of more than one drug) is occasionally used if the disease is advancing rapidly. Combination chemotherapy is similar to that used in aggressive lymphoma; for more information, see the Cancer.Net Guide to Non-Hodgkin Lymphoma.

T-PLL. T-PLL may be treated with drugs that include fludarabine, chlorambucil (Ambochlorin, Leukeran), cyclophosphamide, doxorubicin hydrochloride (Adriamycin, Rubex), vincristine (Oncovin), pentostatin (Nipent), and prednisone. Alemtuzumab (Campath) is a monoclonal antibody directed against a protein called CD52 which is found on the surface of certain T cells and has shown promising results in some patients with T-PLL.

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. Zidovudine is an antiviral therapy used in the treatment of human immunodeficiency virus (HIV). This therapy aims at strengthening the immune system and treating the human T-cell leukemia virus (HTLV). The lymphoma phase is best treated with combination chemotherapy.

Sezary syndrome. Sezary syndrome may be treated using therapies focused on the skin and systemic whole-body therapies. Skin therapies include topical agents (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 body. Systemic therapies for Sezary syndrome include chemotherapy, oral bexarotene (Targretin) (a drug that is similar to vitamin A), denileukin diftitox (Ontak), alpha interferon, and occasionally stem cell transplantation. Alemtuzumab can also be somewhat effective for treating this disease. Recently, a drug, vorinostat (Zolinza), was approved by the U.S. Food and Drug Administration (FDA) cutaneous T-cell lymphoma not responding to other treatments. The choice of treatment depends on the extent of the disease and other factors.

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: During Treatment.