Treatment

The treatment of HCL or PLL depends on whether the disease is causing symptoms, the extent to which the cancer has spread, and the patient’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.

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.

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. Treatment options for B-cell leukemia include chemotherapy, monoclonal antibodies, surgery, stem cell transplantation, and biologic therapy. Each option is described below in more detail.

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.

Drugs that are used to treat HCL include cladribine (Leustatin), pentostatin (Nipent), and recombinant interferon alpha (Alferon N, Intron A, Roferon-A,). In most people with HCL, both cladribine and pentostatin produce remissions that last for many years and are the standard initial therapy for the disease. If the initial response period was long, these drugs can be successful again in treating HCL if it returns (comes back after treatment).

PLL is treated with combination chemotherapy, meaning more than one drug is used. Several regimens may be used. The regimens are selected according to the type of chemotherapy the patient may have received previously and any associated side effects.

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.

Monoclonal antibodies

Monoclonal antibodies are antibodies directed against specific proteins on the cell surface of the cancer cell. Rituximab (Rituxan) is an antibody directed against the surface protein CD20. BL22 immunotoxin is an antibody attached to a toxin that is designed to attach to the surface protein, CD22, and deliver the toxin to the cancer cell. Both of these have been used in the treatment of HCL that did not go into remission after initial therapy (called refractory or resistant HCL).However, BL22 is not approved by the U.S. Food and Drug Administration (FDA) for the treatment of PLL and is only available in clinical trials. Alemtuzumab (Campath) is an antibody directed against the surface protein CD52 and has been used in the treatment of PLL.

Surgery

Surgery to remove the spleen (splenectomy), which also produces white blood cells, may be appropriate in some people.

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.

Transplantation is a higher-risk procedure and is not used frequently in people with B-cell leukemia because it is not a consistently effective therapy for these diseases 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). 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. ALLO transplantation is sometimes considered for younger patients with PLL when chemotherapy was not effective.

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. AUTO transplantation is rarely considered for patients with PLL or HCL.

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

Biologic therapy

Biologic therapy, or immunotherapy, is designed to boost the body’s natural defense to fight the cancer. It uses materials either made by the body or in a laboratory to bolster, target, and restore immune system function.

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