Hairy Cell Leukemia Treatment (PDQ®)

As a National Cancer Institute (NCI)-designated Comprehensive Cancer Center, a core part of our mission is to educate patients and the community about cancer. The following summary is trusted information from the NCI.

General Information About Hairy Cell Leukemia

Hairy cell leukemia is a cancer of the blood and bone marrow. This rare type of leukemia gets worse slowly or does not get worse at all. The disease is called hairy cell leukemia because the leukemia cells look "hairy" when viewed under a microscope.

Normally, the bone marrow makes blood stem cells (immature cells) that develop into mature blood cells over time. A blood stem cell may become a myeloid stem cell or a lymphoid stem cell.

The myeloid stem cell develops into one of three types of mature blood cells:

The lymphoid stem cell develops into a lymphoblast cell and then into one of three types of lymphocytes (white blood cells):

Blood cell development. A blood stem cell goes through several steps to become a red blood cell, platelet, or white blood cell.

In hairy cell leukemia, too many blood stem cells develop into lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. They may also be called leukemic cells. The leukemic cells can build up in the blood and bone marrow so there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. Some of the leukemia cells may collect in the spleen and cause it to swell.

This summary is about hairy cell leukemia. See the following PDQ summaries for information about other types of leukemia:

Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. The cause of hairy cell leukemia is unknown. It occurs more often in older men.

These and other symptoms may be caused by hairy cell leukemia. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

  • Weakness or feeling tired.
  • Fever or frequent infections.
  • Easy bruising or bleeding.
  • Shortness of breath.
  • Weight loss for no known reason.
  • Pain or a feeling of fullness below the ribs.
  • Painless lumps in the neck, underarm, stomach, or groin.

The following tests and procedures may be used:

The treatment options may depend on the following:

  • The number of hairy (leukemia) cells and healthy blood cells in the blood and bone marrow.
  • Whether the spleen is swollen.
  • Whether there are symptoms of leukemia, such as infection.
  • Whether the leukemia has recurred (come back) after previous treatment.

The prognosis (chance of recovery) depends on the following:

  • Whether the hairy cell leukemia does not grow or grows so slowly it does not need treatment.
  • Whether the hairy cell leukemia responds to treatment.

Treatment often results in a long-lasting remission (a period during which some or all of the signs and symptoms of the leukemia are gone). If the leukemia returns after it has been in remission, retreatment often causes another remission.

Stages of Hairy Cell Leukemia

Staging is the process used to find out how far the cancer has spread. Groups are used in place of stages for hairy cell leukemia. The disease is grouped as untreated, progressive, or refractory.

Untreated hairy cell leukemia

The hairy cell leukemia is newly diagnosed and has not been treated except to relieve symptoms such as weight loss and infections. In untreated hairy cell leukemia, some or all of the following conditions occur:

Progressive hairy cell leukemia

In progressive hairy cell leukemia, the leukemia has been treated with either chemotherapy or splenectomy (removal of the spleen) and one or both of the following conditions occur:

When cancer cells spread outside the blood, a solid tumor may form. This process is called metastasis. The three ways that cancer cells spread in the body are:

  • Through the blood. Cancer cells travel through the blood, invade solid tissues in the body, such as the brain or heart, and form a solid tumor.
  • Through the lymph system. Cancer cells invade the lymph system, travel through the lymph vessels, and form a solid tumor in other parts of the body.
  • Through solid tissue. Cancer cells that have formed a solid tumor spread to tissues in the surrounding area.

The new (metastatic) tumor is the same type of cancer as the primary cancer. For example, if leukemia cells spread to the brain, the cancer cells in the brain are actually leukemia cells. The disease is metastatic leukemia, not brain cancer.

Relapsed or Refractory Hairy Cell Leukemia

Relapsed hairy cell leukemia has come back after treatment. Refractory hairy cell leukemia has not responded to treatment.

Treatment Option Overview

Different types of treatment are available for patients with hairy cell leukemia. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Watchful waiting is closely monitoring a patient's condition, without giving any treatment until symptoms appear or change.

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated. Cladribine and pentostatin are anticancer drugs commonly used to treat hairy cell leukemia. These drugs may increase the risk of developing other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important.

Biologic therapy is a cancer treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Interferon alfa is a biologic agent commonly used to treat hairy cell leukemia.

Splenectomy is a surgical procedure to remove the spleen.

Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy is a type of targeted therapy being studied in the treatment of hairy cell leukemia.

Monoclonal antibody therapy uses antibodies made in the laboratory from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells.

A monoclonal antibody called rituximab may be used for certain patients with hairy cell leukemia.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Hairy Cell Leukemia

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Untreated Hairy Cell Leukemia

If the patient's blood cell counts are not too low and there are no symptoms, treatment may not be needed and the patient is carefully watched for changes in his or her condition. If blood cell counts become too low or symptoms appear, initial treatment may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with untreated hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Progressive Hairy Cell Leukemia

Treatment for progressive hairy cell leukemia may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with progressive hairy cell leukemia, initial treatment. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

Relapsed or Refractory Hairy Cell Leukemia

Treatment of relapsed or refractory hairy cell leukemia may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with refractory hairy cell leukemia. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.

To Learn More About Hairy Cell Leukemia

For more information from the National Cancer Institute about hairy cell leukemia, see the following:

For general cancer information and other resources from the National Cancer Institute, see the following:



Cancer Care Services

Clinical Trials