Leukaemia

Leukemia

Definition of Leukemia : Cancer that starts in blood-forming tissue, such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the bloodstream.

On this page:
Adult Acute Lymphoblastic Leukaemia
Adult Acute Myeloid Leukaemia
Chronic Lymphocytic Leukaemia
Chronic Myelogenous Leukaemia
Hairy Cell Leukaemia

General Information about Adult Acute Lymphoblastic Leukaemia

Adult acute lymphoblastic leukaemia (ALL) is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).

Adult acute lymphoblastic leukaemia (ALL; also called acute lymphocytic leukaemia) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated.

Leukaemia may affect red blood cells, white blood cells, and platelets.

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

A myeloid stem cell becomes one of three types of mature blood cells:

  • Red blood cells that carry oxygen and other substances to all tissues of the body.
  • Platelets that form blood clots to stop bleeding.
  • Granulocytes (white blood cells) that fight infection and disease.

A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells):

  • B lymphocytes that make antibodies to help fight infection.
  • T lymphocytes that help B lymphocytes make the antibodies that help fight infection.
  • Natural killer cells that attack cancer cells and viruses.

In ALL, too many stem cells become lymphoblasts, B lymphocytes, or T lymphocytes. These cells are also called leukaemia cells. These leukaemia cells are not able to fight infection very well. Also, as the number of leukaemia cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding. The cancer can also spread to the central nervous system (brain and spinal cord)..

Previous chemotherapy and exposure to radiation may increase the risk of developing ALL.

Anything that increases your risk 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. Talk with your doctor if you think you may be at risk. Possible risk factors for ALL include the following:

  • Being male.
  • Being white.
  • Being older than 70.
  • Past treatment with chemotherapy or radiation therapy.
  • Being exposed to radiation from an atomic bomb.
  • Having certain genetic disorders, such as Down syndrome.

Signs and symptoms.

The early signs and symptoms of ALL may be like the flu or other common diseases. Check with your doctor if you have any of the following:

  • Weakness or feeling tired.
  • Fever or night sweats.
  • Easy bruising or bleeding.
  • Petechiae (flat, pinpoint spots under the skin, caused by bleeding).
  • Shortness of breath.
  • Weight loss or loss of appetite.
  • Pain in the bones or stomach.
  • Pain or feeling of fullness below the ribs.
  • Painless lumps in the neck, underarm, stomach, or groin.
  • Having many infections.

These and other signs and symptoms may be caused by adult acute lymphoblastic leukaemia or by other conditions.

Tests used to detect and diagnose adult acute lymphoblastic leukaemia.

The following tests and procedures may be used:

  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as infection or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells and platelets.
    • The number and type of white blood cells.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the blood sample made up of red blood cells.
  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
  • Peripheral blood smear : A procedure in which a sample of blood is checked for blast cells, the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells.
  • Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells.

The following tests may be done on the samples of blood or bone marrow tissue that are removed:

  • Cytogenetic analysis: A laboratory test in which the cells in a sample of blood or bone marrow are looked at under a microscope to find out if there are certain changes in the chromosomes in the lymphocytes. For example, sometimes in ALL, part of one chromosome is moved to another chromosome. This is called the Philadelphia chromosome. Other tests, such as fluorescence in situ hybridization (FISH), may also be done to look for certain changes in the chromosomes.
  • Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose the subtype of ALL by comparing the cancer cells to normal cells of the immune system. For example, a cytochemistry study may test the cells in a sample of tissue using chemicals (dyes) to look for certain changes in the sample. A chemical may cause a color change in one type of leukaemia cell but not in another type of leukaemia cell.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The age of the patient.
  • Whether the cancer has spread to the brain or spinal cord.
  • Whether there are certain changes in the genes, including the Philadelphia chromosome.
  • Whether the cancer has been treated before or has recurred (come back).

Stages of Adult Acute Lymphoblastic Leukaemia

Once adult ALL has been diagnosed, tests are done to find out if the cancer has spread to the central nervous system (brain and spinal cord) or to other parts of the body.

The extent or spread of cancer is usually described as stages. It is important to know whether the leukaemia has spread outside the blood and bone marrow in order to plan treatment. The following tests and procedures may be used to determine if the leukaemia has spread:

  • Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
  • Lumbar puncture : A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

There is no standard staging system for adult ALL.

The disease is described as untreated, in remission, or recurrent.

Untreated adult ALL

The ALL is newly diagnosed and has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain.

  • The complete blood count is abnormal.
  • More than 5% of the cells in the bone marrow are blasts (leukaemia cells).
  • There are signs and symptoms of leukaemia.

Adult ALL in remission

The ALL has been treated.

  • The complete blood count is normal.
  • 5% or fewer of the cells in the bone marrow are blasts (leukaemia cells).
  • There are no signs or symptoms of leukaemia other than in the bone marrow.

Recurrent Adult Acute Lymphoblastic Leukaemia

Recurrent adult acute lymphoblastic leukaemia (ALL) is cancer that has recurred (come back) after going into remission. The ALL may come back in the blood, bone marrow, or other parts of the body.

Treatment Option Overview

There are different types of treatment for patients with adult ALL.

Different types of treatment are available for patients with adult acute lymphoblastic leukaemia (ALL). 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.

The treatment of adult ALL usually has two phases.

The treatment of adult ALL is done in phases:

  • Remission induction therapy: This is the first phase of treatment. The goal is to kill the leukaemia cells in the blood and bone marrow. This puts the leukaemia into remission.
  • Post-remission therapy: This is the second phase of treatment. It begins once the leukaemia is in remission. The goal of post-remission therapy is to kill any remaining leukaemia cells that may not be active but could begin to regrow and cause a relapse. This phase is also called remission continuation therapy.

Treatment called central nervous system (CNS) sanctuary therapy is usually given during each phase of therapy. Because standard doses of chemotherapy may not reach leukaemia cells in the CNS (brain and spinal cord), the cells are able to “find sanctuary” (hide) in the CNS. High doses of certain anticancer drugs, intrathecal chemotherapy, and radiation therapy to the brain are able to reach leukaemia cells in the CNS. They are given to kill the leukaemia cells and keep the cancer from recurring (coming back). CNS sanctuary therapy is also called CNS prophylaxis.

Four types of standard treatment are used:

Chemotherapy

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 (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Intrathecal chemotherapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used to prevent cancer from spreading to the brain and spinal cord, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy.

External radiation therapy uses a machine outside the body to send radiation toward the cancer.

Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

The way the radiation therapy is given depends on the type of cancer. External radiation therapy may be used to treat adult ALL that has spread, or may spread, to the brain and spinal cord. When used this way, it is called central nervous system (CNS) sanctuary therapy or CNS prophylaxis. External radiation therapy may also be used as palliative therapy to relieve symptoms and improve quality of life.

Chemotherapy with stem cell transplant

Stem cell transplant is a method of giving chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy and tyrosine kinase inhibitor therapy are types of targeted therapy used to treat adult ALL.

Monoclonal antibody therapy is a cancer treatment that 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. Blinatumomab and inotuzumab ozogamicin are monoclonal antibodies used with stem cell transplant to treat adult ALL.

Tyrosine kinase inhibitor therapy blocks the enzyme, tyrosine kinase, that causes stem cells to develop into more white blood cells (blasts) than the body needs. Imatinib mesylate (Gleevec), dasatinib, and nilotinib are tyrosine kinase inhibitors used to treat adult ALL.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied.

Biologic therapy

Biologic therapy is a 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.

Chimeric antigen receptor (CAR) T-cell therapy

CAR T-cell therapy is a type of immunotherapy that changes the patient’s T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient’s blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of adult ALL that has recurred (come back).

Patients may want to think about taking part in a clinical trial.

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.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

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.

Patients with ALL may have late effects after treatment.

Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of treatment for ALL may include the risk of second cancers (new types of cancer). Regular follow-up exams are very important for long-term survivors.

Treatment for adult acute lymphoblastic leukemia may cause side effects.

Follow-up tests may be needed.

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 Adult Acute Lymphoblastic Leukaemia

Untreated Adult Acute Lymphoblastic Leukaemia

Standard treatment of adult acute lymphoblastic leukaemia (ALL) during the remission induction phase includes the following:

  • Combination chemotherapy.
  • Tyrosine kinase inhibitor therapy with imatinib mesylate, in certain patients. Some of these patients will also have combination chemotherapy.
  • Supportive care including antibiotics and red blood cell and platelet transfusions.
  • CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain.

Adult Acute Lymphoblastic Leukaemia in Remission

Standard treatment of adult ALL during the post-remission phase includes the following:

  • Chemotherapy.
  • Tyrosine kinase inhibitor therapy.
  • Chemotherapy with stem cell transplant.
  • CNS prophylaxis therapy including chemotherapy (intrathecal and/or systemic) with or without radiation therapy to the brain.

Recurrent Adult Acute Lymphoblastic Leukaemia

Standard treatment of recurrent adult ALL may include the following:

  • Combination chemotherapy followed by stem cell transplant.
  • Monoclonal antibody therapy (blinatumomab or inotuzumab ozogamicin) followed by stem cell transplant.
  • Low-dose radiation therapy as palliative care to relieve symptoms and improve the quality of life.
  • Tyrosine kinase inhibitor therapy with dasatinib for certain patients.

Some of the treatments being studied in clinical trials for recurrent adult ALL include the following:

  • A clinical trial of stem cell transplant using the patient’s stem cells.
  • A clinical trial of biologic therapy.
  • A clinical trial of new anticancer drugs.


Updated:
June 1, 2018
Source: National Cancer Institute



General Information About Adult Acute Myeloid Leukemia

Adult acute myeloid leukaemia (AML) is a type of cancer in which the bone marrow makes abnormal myeloblasts (a type of white blood cell), red blood cells, or platelets.

Adult acute myeloid leukaemia (AML) is a cancer of the blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated. It is the most common type of acute leukaemia in adults. AML is also called acute myelogenous leukaemia, acute myeloblastic leukaemia, acute granulocytic leukaemia, and acute nonlymphocytic leukaemia.

Leukaemia may affect red blood cells, white blood cells, and platelets.

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

A myeloid stem cell becomes one of three types of mature blood cells:

  • Red blood cells that carry oxygen and other substances to all tissues of the body.
  • White blood cells that fight infection and disease.
  • Platelets that form blood clots to stop bleeding.

In AML, the myeloid stem cells usually become a type of immature white blood cell called myeloblasts (or myeloid blasts). The myeloblasts in AML are abnormal and do not become healthy white blood cells. Sometimes in AML, too many stem cells become abnormal red blood cells or platelets. These abnormal white blood cells, red blood cells, or platelets are also called leukaemia cells or blasts. Leukaemia cells can build up in the bone marrow and blood so there is less room for healthy white blood cells, red blood cells, and platelets. When this happens, infection, anemia, or easy bleeding may occur. The leukaemia cells can spread outside the blood to other parts of the body, including the central nervous system (brain and spinal cord), skin, and gums.

There are different subtypes of AML.

Most AML subtypes are based on how mature (developed) the cancer cells are at the time of diagnosis and how different they are from normal cells.

Acute promyelocytic leukaemia (APL) is a subtype of AML that occurs when parts of two genes stick together. APL usually occurs in middle-aged adults. Signs of APL may include both bleeding and forming blood clots.

Smoking, previous chemotherapy treatment, and exposure to radiation may affect the risk of adult AML.

Anything that increases your risk 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. Talk with your doctor if you think you may be at risk. Possible risk factors for AML include the following:

  • Being male.
  • Smoking, especially after age 60.
  • Having had treatment with chemotherapy or radiation therapy in the past.
  • Having had treatment for childhood acute lymphoblastic leukaemia (ALL) in the past.
  • Being exposed to radiation from an atomic bomb or to the chemical benzene.
  • Having a history of a blood disorder such as myelodysplastic syndrome.

Signs and Symptoms of adult acute myeloid leukaemia

The early signs and symptoms of AML may be like those caused by the flu or other common diseases. Check with your doctor if you have any of the following:

  • Fever.
  • Shortness of breath.
  • Easy bruising or bleeding.
  • Petechiae (flat, pinpoint spots under the skin caused by bleeding).
  • Weakness or feeling tired.
  • Weight loss or loss of appetite.

Tests used to detect and diagnose adult acute myeloid leukaemia

The following tests and procedures may be used:

  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells, white blood cells, and platelets.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the sample made up of red blood cells.
  • Peripheral blood smear : A procedure in which a sample of blood is checked for blast cells, the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells.
  • Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
  • Cytogenetic analysis : A laboratory test in which the cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes. Other tests, such as fluorescence in situ hybridization (FISH), may also be done to look for certain changes in the chromosomes.
  • Immunophenotyping : A process used to identify cells, based on the types of antigens or markers on the surface of the cell. This process is used to diagnose the subtype of AML by comparing the cancer cells to normal cells of the immune system. For example, a cytochemistry study may test the cells in a sample of tissue using chemicals (dyes) to look for certain changes in the sample. A chemical may cause a color change in one type of leukaemia cell but not in another type of leukaemia cell.
  • Reverse transcription – polymerase chain reaction test (RT–PCR): A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes. This test is used to diagnose certain types of AML including acute promyelocytic leukaemia (APL).

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on:

  • The age of the patient.
  • The subtype of AML.
  • Whether the patient received chemotherapy in the past to treat a different cancer.
  • Whether there is a history of a blood disorder such as myelodysplastic syndrome.
  • Whether the cancer has spread to the central nervous system.
  • Whether the cancer has been treated before or recurred (come back).

It is important that acute leukaemia be treated right away.

Stages of Adult Acute Myeloid Leukaemia

Key Points for This Section

  • Once adult acute myeloid leukaemia (AML) has been diagnosed, tests are done to find out if the cancer has spread to other parts of the body.
  • There is no standard staging system for adult AML.

Once adult acute myeloid leukaemia (AML) has been diagnosed, tests are done to find out if the cancer has spread to other parts of the body.

The extent or spread of cancer is usually described as stages. In adult acute myeloid leukaemia (AML), the subtype of AML and whether the leukaemia has spread outside the blood and bone marrow are used instead of the stage to plan treatment. The following tests and procedures may be used to determine if the leukaemia has spread:

  • Lumbar puncture : A procedure used to collect cerebrospinal fluid from the spinal column. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of the abdomen, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

There is no standard staging system for adult AML.

The disease is described as untreated, in remission, or recurrent.

Untreated adult AML

In untreated adult AML, the disease is newly diagnosed. It has not been treated except to relieve signs and symptoms such as fever, bleeding, or pain, and the following are true:

  • The complete blood count is abnormal.
  • At least 20% of the cells in the bone marrow are blasts (leukaemia cells).
  • There are signs or symptoms of leukaemia.

Adult AML in remission

In adult AML in remission, the disease has been treated and the following are true:

  • The complete blood count is normal.
  • Less than 5% of the cells in the bone marrow are blasts (leukaemia cells).
  • There are no signs or symptoms of leukaemia in the brain and spinal cord or elsewhere in the body.

Recurrent Adult AML

Recurrent AML is cancer that has recurred (come back) after it has been treated. The AML may come back in the blood or bone marrow.

Treatment Option Overview

There are different types of treatment for patients with adult acute myeloid leukaemia.

Different types of treatment are available for patients with adult acute myeloid leukaemia (AML). 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.

The treatment of adult AML usually has 2 phases.

The 2 treatment phases of adult AML are:

  • Remission induction therapy: This is the first phase of treatment. The goal is to kill the leukaemia cells in the blood and bone marrow. This puts the leukaemia into remission.
  • Post-remission therapy: This is the second phase of treatment. It begins after the leukaemia is in remission. The goal of post-remission therapy is to kill any remaining leukaemia cells that may not be active but could begin to regrow and cause a relapse. This phase is also called remission continuation therapy.

Four types of standard treatment are used:

Chemotherapy

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 (intrathecal chemotherapy), an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Intrathecal chemotherapy may be used to treat adult AML that has spread, or may spread to the brain and spinal cord. Combination chemotherapy is treatment using more than one anticancer drug.

The way the chemotherapy is given depends on the subtype of the cancer being treated and whether it has spread to the brain and spinal cord.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:

  • External radiation therapy uses a machine outside the body to send radiation toward the cancer.
  • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Stem cell transplant

Stem cell transplant is a method of giving chemotherapy and replacing blood -forming cells that are abnormal or destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI website.

Targeted therapy

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

Monoclonal antibody therapy is a cancer treatment that 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.

Treatment for adult acute myeloid leukemia may cause side effects.

For information about side effects caused by treatment for cancer, see our Side Effects page.

Patients may want to think about taking part in a clinical trial.

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.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

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.

Follow-up tests may be needed.

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 Adult Acute Myeloid Leukaemia

Untreated Adult Acute Myeloid Leukaemia

Standard treatment of untreated adult acute myeloid leukaemia (AML) during the remission induction phase depends on the subtype of AML and may include the following:

  • Combination chemotherapy.
  • High-dose combination chemotherapy.
  • Low-dose chemotherapy.
  • Intrathecal chemotherapy.
  • All-trans retinoic acid (ATRA) plus chemotherapy for the treatment of acute promyelocytic leukaemia (APL).
  • A clinical trial of arsenic trioxide and ATRA and combination chemotherapy for the treatment of APL.

Adult Acute Myeloid Leukaemia in Remission

Standard treatment of adult AML during the remission phase depends on the subtype of AML and may include the following:

  • Combination chemotherapy.
  • High-dose chemotherapy, with or without radiation therapy, and stem cell transplant using the patient’s stem cells.
  • High-dose chemotherapy and stem cell transplant using donor stem cells.
  • A clinical trial of arsenic trioxide.

Recurrent Adult Acute Myeloid Leukaemia

There is no standard treatment for recurrent adult AML. Treatment depends on the subtype of AML and may include the following:

  • Combination chemotherapy.
  • Targeted therapy with monoclonal antibodies.
  • Stem cell transplant.
  • Arsenic trioxide therapy.
  • A clinical trial of arsenic trioxide therapy followed by stem cell transplant.

Updated: May 18, 2018
Source: National Cancer Institute

Chronic Lymphocytic Leukaemia

Chronic lymphocytic leukaemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).

Chronic lymphocytic leukaemia (also called CLL) is a blood and bone marrow disease that usually gets worse slowly. CLL is one of the most common types of leukaemia in adults. It often occurs during or after middle age; it rarely occurs in children.

Leukaemia may affect red blood cells, white blood cells, and platelets.

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

A myeloid stem cell becomes one of three types of mature blood cells:

  • Red blood cells that carry oxygen and other substances to all tissues of the body.
  • White blood cells that fight infection and disease.
  • Platelets that form blood clots to stop bleeding.

A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells):

  • B lymphocytes that make antibodies to help fight infection.
  • T lymphocytes that help B lymphocytes make antibodies to fight infection.
  • Natural killer cells that attack cancer cells and viruses.

In CLL, too many blood stem cells become abnormal lymphocytes and do not become healthy white blood cells. The abnormal lymphocytes may also be called leukaemia cells. The lymphocytes are not able to fight infection very well. Also, as the number of lymphocytes increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may cause infection, anemia, and easy bleeding.

Older age can affect the risk of developing chronic lymphocytic leukaemia.

Anything that increases your risk 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. Talk with your doctor if you think you may be at risk. Risk factors for CLL include the following:

  • Being middle-aged or older, male, or white.
  • A family history of CLL or cancer of the lymph system.
  • Having relatives who are Russian Jews or Eastern European Jews.

Signs and symptoms of chronic lymphocytic leukaemia.

Usually CLL does not cause any signs or symptoms and is found during a routine blood test. Signs and symptoms may be caused by CLL or by other conditions. Check with your doctor if you have any of the following:

  • Painless swelling of the lymph nodes in the neck, underarm, stomach, or groin.
  • Feeling very tired.
  • Pain or fullness below the ribs.
  • Fever and infection.
  • Weight loss for no known reason.

Tests used to detect and diagnose chronic lymphocytic leukaemia.

The following tests and procedures may be used:

  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells and platelets.
    • The number and type of white blood cells.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the blood sample made up of red blood cells.
  • Immunophenotyping : A laboratory test in which the antigens or markers on the surface of a blood or bone marrow cell are checked to see if they are lymphocytes or myeloid cells. If the cells are malignant lymphocytes (cancer), they are checked to see if they are B lymphocytes or T lymphocytes.
  • FISH (fluorescence in situ hybridization): A laboratory technique used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA bind to specific genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light.
  • Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.
  • IgVH gene mutation test: A laboratory test done on a bone marrow or blood sample to check for an IgVH gene mutation. Patients with an IgVH gene mutation have a better prognosis.
  • Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells.

Certain factors affect treatment options and prognosis (chance of recovery).

Treatment options depend on:

  • The stage of the disease.
  • Red blood cell, white blood cell, and platelet blood counts.
  • Whether there are signs or symptoms, such as fever, chills, or weight loss.
  • Whether the liver, spleen, or lymph nodes are larger than normal.
  • The response to initial treatment.
  • Whether the CLL has recurred (come back).

The prognosis (chance of recovery) depends on:

  • Whether there is a change in the DNA and the type of change, if there is one.
  • Whether lymphocytes are spread throughout the bone marrow.
  • The stage of the disease.
  • Whether the CLL gets better with treatment or has recurred (come back).
  • Whether the CLL progresses to lymphoma or prolymphocytic leukaemia.
  • The patient’s general health.

After chronic lymphocytic leukaemia has been diagnosed, tests are done to find out how far the cancer has spread in the blood and bone marrow.

Staging is the process used to find out how far the cancer has spread. It is important to know the stage of the disease in order to plan the best treatment. The following tests may be used in the staging process:

  • Chest x-ray : An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body, such as the lymph nodes.
  • MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain and spinal cord. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • PET-CT scan : A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time with the same machine. The combined scans give more detailed pictures of areas inside the body than either scan gives by itself. A PET scan is a procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
  • Antiglobulin test : A test in which a sample of blood is looked at under a microscope to find out if there are any antibodies on the surface of red blood cells or platelets. These antibodies may react with and destroy the red blood cells and platelets. This test is also called a Coombs test.

The following stages are used for chronic lymphocytic leukaemia:

Stage 0

In stage 0 chronic lymphocytic leukaemia, there are too many lymphocytes in the blood, but there are no other signs or symptoms of leukaemia. Stage 0 chronic lymphocytic leukaemia is indolent (slow-growing).

Stage I

In stage I chronic lymphocytic leukaemia, there are too many lymphocytes in the blood and the lymph nodes are larger than normal.

Stage II

In stage II chronic lymphocytic leukaemia, there are too many lymphocytes in the blood, the liver or spleen is larger than normal, and the lymph nodes may be larger than normal.

Stage III

In stage III chronic lymphocytic leukaemia, there are too many lymphocytes in the blood and there are too few red blood cells. The lymph nodes, liver, or spleen may be larger than normal.

Stage IV

In stage IV chronic lymphocytic leukaemia, there are too many lymphocytes in the blood and too few platelets. The lymph nodes, liver, or spleen may be larger than normal and there may be too few red blood cells.

Refractory Chronic Lymphocytic Leukaemia

Refractory chronic lymphocytic leukaemia is cancer that does not get better with treatment.

Treatment Option Overview

There are different types of treatment for patients with chronic lymphocytic leukaemia.

Different types of treatment are available for patients with chronic lymphocytic leukaemia. 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.

Five types of standard treatment are used:

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until signs or symptoms appear or change. This is also called observation. During this time, problems caused by the disease, such as infection, are treated.

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:

  • External radiation therapy uses a machine outside the body to send radiation toward the cancer.
  • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Chemotherapy

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, or 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.

Surgery

Splenectomy is surgery to remove the spleen.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Monoclonal antibody therapy and tyrosine kinase inhibitor therapy are types of targeted therapy used in the treatment of chronic lymphocytic leukaemia.

Monoclonal antibody therapy is a cancer treatment that 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 in the body 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.

Tyrosine kinase inhibitor therapy is a cancer treatment that blocks signals needed for tumors to grow.

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. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

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.

New types of treatment are being tested in clinical trials.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied.

Chemotherapy with stem cell transplant

Chemotherapy with stem cell transplant is a method of giving chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

Biologic therapy

Biologic therapy is a 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.

Chimeric antigen receptor (CAR) T-cell therapy

CAR T-cell therapy is a type of immunotherapy that changes the patient’s T cells (a type of immune system cell) so they will attack certain proteins on the surface of cancer cells. T cells are taken from the patient and special receptors are added to their surface in the laboratory. The changed cells are called chimeric antigen receptor (CAR) T cells. The CAR T cells are grown in the laboratory and given to the patient by infusion. The CAR T cells multiply in the patient’s blood and attack cancer cells. CAR T-cell therapy is being studied in the treatment of chronic lymphocytic leukemia.

Follow-up tests may be needed.

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 by Stage

Treatment of stage 0 chronic lymphocytic leukemia is usually watchful waiting.

Treatment of stage I, stage II, stage III, and stage IV chronic lymphocytic leukemia may include the following:

  • Watchful waiting when there are few or no signs or symptoms.
  • Targeted therapy with a monoclonal antibody, a tyrosine kinase inhibitor, or a BCL2 inhibitor.
  • Chemotherapy with 1 or more drugs, with or without steroids or monoclonal antibody therapy.
  • Low-dose external radiation therapy to areas of the body where cancer is found, such as the spleen or lymph nodes.
  • A clinical trial of chemotherapy and biologic therapy with stem cell transplant.
  • A clinical trial of a new treatment.

Treatment Options for Refractory Chronic Lymphocytic Leukemia

Treatment of refractory chronic lymphocytic leukemia may include the following:

  • Repeat treatment with monoclonal antibody therapy and combination chemotherapy.
  • A clinical trial of chemotherapy with stem cell transplant.
  • A clinical trial of a new treatment.

Updated: May 18, 2018
Source: National Cancer Institute


General Information About Chronic Myelogenous Leukemia

Chronic myelogenous leukaemia is a disease in which the bone marrow makes too many white blood cells.

Chronic myelogenous leukaemia (also called CML or chronic granulocytic leukaemia) is a slowly progressing blood and bone marrow disease that usually occurs during or after middle age, and rarely occurs in children.

Leukaemia may affect red blood cells, white blood cells, and platelets.

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

A myeloid stem cell becomes one of three types of mature blood cells:

  • Red blood cells that carry oxygen and other substances to all tissues of the body.
  • Platelets that form blood clots to stop bleeding.
  • Granulocytes (white blood cells) that fight infection and disease.

In CML, too many blood stem cells become a type of white blood cell called granulocytes. These granulocytes are abnormal and do not become healthy white blood cells. They are also called leukaemia cells. The leukaemia 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. When this happens, infection, anemia, or easy bleeding may occur.

Signs and symptoms of chronic myelogenous leukaemia.

These and other signs and symptoms may be caused by CML or by other conditions. Check with your doctor if you have any of the following:

  • Feeling very tired.
  • Weight loss for no known reason.
  • Night sweats.
  • Fever.
  • Pain or a feeling of fullness below the ribs on the left side.

Sometimes CML does not cause any symptoms at all.

Most people with CML have a gene mutation (change) called the Philadelphia chromosome.

Every cell in the body contains DNA (genetic material) that determines how the cell looks and acts. DNA is contained inside chromosomes. In CML, part of the DNA from one chromosome moves to another chromosome. This change is called the “ Philadelphia chromosome.” It results in the bone marrow making an enzyme, called tyrosine kinase, that causes too many stem cells to become white blood cells (granulocytes or blasts).

The Philadelphia chromosome is not passed from parent to child.

Tests used to detect and diagnose chronic myelogenous leukaemia.

The following tests and procedures may be used:

  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease such as an enlarged spleen. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Complete blood count (CBC) with differential : A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells and platelets.
    • The number and type of white blood cells.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the blood sample made up of red blood cells.
  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
  • Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for abnormal cells. One of the following tests may be done on the samples of blood or bone marrow tissue that are removed:
  • Cytogenetic analysis: A test in which cells in a sample of blood or bone marrow are viewed under a microscope to look for certain changes in the chromosomes, such as the Philadelphia chromosome.
  • FISH (fluorescence in situ hybridization): A laboratory technique used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA bind to specific genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light.
  • Reverse transcription polymerase chain reaction test (RT–PCR): A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on the following:

  • The patient’s age.
  • The phase of CML.
  • The amount of blasts in the blood or bone marrow.
  • The size of the spleen at diagnosis.
  • The patient’s general health.

Stages of Chronic Myelogenous Leukaemia

After chronic myelogenous leukaemia has been diagnosed, tests are done to find out if the cancer has spread.

Staging is the process used to find out how far the cancer has spread. There is no standard staging system for chronic myelogenous leukaemia (CML). Instead, the disease is classified by phase: chronic phase, accelerated phase, or blastic phase. It is important to know the phase in order to plan treatment. The information from tests and procedures done to detect (find) and diagnose chronic myelogenous leukaemia is also used to plan treatment.

Chronic myelogenous leukaemia has 3 phases.

As the amount of blast cells increases in the blood and bone marrow, there is less room for healthy white blood cells, red blood cells, and platelets. This may result in infections, anemia, and easy bleeding, as well as bone pain and pain or a feeling of fullness below the ribs on the left side. The number of blast cells in the blood and bone marrow and the severity of signs or symptoms determine the phase of the disease.

Chronic phase

In chronic phase CML, fewer than 10% of the cells in the blood and bone marrow are blast cells.

Accelerated phase

In accelerated phase CML, 10% to 19% of the cells in the blood and bone marrow are blast cells.

Blastic phase

In blastic phase CML, 20% or more of the cells in the blood or bone marrow are blast cells. When tiredness, fever, and an enlarged spleen occur during the blastic phase, it is called blast crisis.

Relapsed Chronic Myelogenous Leukaemia

In relapsed CML, the number of blast cells increases after a remission.

Treatment Option Overview

There are different types of treatment for patients with chronic myelogenous leukaemia.

Different types of treatment are available for patients with chronic myelogenous leukaemia (CML). 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 about 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.

Six types of standard treatment are used:

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Tyrosine kinase inhibitors are targeted therapy drugs used to treat chronic myelogenous leukaemia.

Imatinib mesylate, nilotinib, and dasatinib are tyrosine kinase inhibitors that may be used as initial treatment for newly diagnosed patients with chronic phase CML.

Chemotherapy

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.

Biologic therapy

Biologic therapy is a 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.

High-dose chemotherapy with stem cell transplant

High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body’s blood cells.

Donor lymphocyte infusion (DLI)

Donor lymphocyte infusion (DLI) is a cancer treatment that may be used after stem cell transplant. Lymphocytes (a type of white blood cell) from the stem cell transplant donor are removed from the donor’s blood and may be frozen for storage. The donor’s lymphocytes are thawed if they were frozen and then given to the patient through one or more infusions. The lymphocytes see the patient’s cancer cells as not belonging to the body and attack them.

Surgery

Splenectomy is surgery to remove the spleen.

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. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

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.

Follow-up tests may be needed.

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 Chronic Myelogenous Leukaemia

Chronic Phase Chronic Myelogenous Leukaemia

Treatment of chronic phase chronic myelogenous leukaemia may include the following:

  • Targeted therapy with a tyrosine kinase inhibitor.
  • High-dose chemotherapy with donor stem cell transplant.
  • Chemotherapy.
  • Splenectomy.
  • A clinical trial of lower-dose chemotherapy with donor stem cell transplant.
  • A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI’s list of cancer clinical trials that are now accepting patients with chronic phase chronic myelogenous leukaemia. 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. Talk with your doctor about clinical trials that may be right for you.

Accelerated Phase Chronic Myelogenous Leukaemia

Treatment of accelerated phase chronic myelogenous leukaemia may include the following:

  • Donor stem cell transplant.
  • Targeted therapy with a tyrosine kinase inhibitor.
  • Tyrosine kinase inhibitor therapy followed by a donor stem cell transplant.
  • Biologic therapy (interferon) with or without chemotherapy.
  • High-dose chemotherapy.
  • Chemotherapy.
  • Transfusion therapy to replace red blood cells, platelets, and sometimes white blood cells, to relieve symptoms and improve quality of life.
  • A clinical trial of a new treatment.

Blastic Phase Chronic Myelogenous Leukaemia

Treatment of blastic phase chronic myelogenous leukaemia may include the following:

  • Targeted therapy with a tyrosine kinase inhibitor.
  • Chemotherapy using one or more drugs.
  • High-dose chemotherapy.
  • Donor stem cell transplant.
  • Chemotherapy as palliative therapy to relieve symptoms and improve quality of life.
  • A clinical trial of a new treatment.

Relapsed Chronic Myelogenous Leukaemia

Treatment of relapsed chronic myelogenous leukaemia may include the following:

  • Targeted therapy with a tyrosine kinase inhibitor.
  • Donor stem cell transplant.
  • Chemotherapy.
  • Donor lymphocyte infusion.
  • Biologic therapy (interferon).
  • A clinical trial of new types or higher doses of targeted therapy or donor stem cell transplant.

Updated: May 18, 2018
Source: National Cancer Institute


General Information About Hairy Cell Leukemia

Hairy cell leukaemia is a type of cancer in which the bone marrow makes too many lymphocytes (a type of white blood cell).

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

Leukaemia may affect red blood cells, white blood cells, and platelets.

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

A myeloid stem cell becomes one of three types of mature blood cells:

  • Red blood cells that carry oxygen and other substances to all tissues of the body.
  • White blood cells that fight infection and disease.
  • Platelets that form blood clots to stop bleeding.

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

  • B lymphocytes that make antibodies to help fight infection.
  • T lymphocytes that help B lymphocytes make antibodies to help fight infection.
  • Natural killer cells that attack cancer cells and viruses.

In hairy cell leukaemia, too many blood stem cells become lymphocytes. These lymphocytes are abnormal and do not become healthy white blood cells. They are also called leukaemia cells. The leukaemia 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 leukaemia cells may collect in the spleen and cause it to swell.

Gender and age may affect the risk of hairy cell leukaemia.

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. Talk with your doctor if you think you may be at risk. The cause of hairy cell leukaemia is unknown. It occurs more often in older men.

Signs and symptoms of hairy cell leukaemia.

These and other signs and symptoms may be caused by hairy cell leukaemia or by other conditions. Check with your doctor if you have any of the following:

  • 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.

Tests that examine the blood and bone marrow are used to detect (find) and diagnose hairy cell leukaemia.

The following tests and procedures may be used:

  • Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as a swollen spleen, lumps, or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
    • The number of red blood cells, white blood cells, and platelets.
    • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
    • The portion of the sample made up of red blood cells.
  • Peripheral blood smear : A procedure in which a sample of blood is checked for cells that look “hairy,” the number and kinds of white blood cells, the number of platelets, and changes in the shape of blood cells.
  • Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
  • Bone marrow aspiration and biopsy : The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone or breastbone. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
  • Immunophenotyping : A laboratory test in which the antigens or markers on the surface of a blood or bone marrow cell are checked to see what type of cell it is. This test is done to diagnose the specific type of leukaemia by comparing the cancer cells to normal cells of the immune system.
  • Flow cytometry : A laboratory test that measures the number of cells in a sample, the percentage of live cells in a sample, and certain characteristics of cells, such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained with a light-sensitive dye, placed in a fluid, and passed in a stream before a laser or other type of light. The measurements are based on how the light-sensitive dye reacts to the light.
  • Gene mutation test: A laboratory test done on a bone marrow or blood sample to check for mutations in the BRAF gene. A BRAF gene mutation is often found in patients with hairy cell leukemia.
  • Cytogenetic analysis : A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography. A CT scan of the abdomen may be done to check for swollen lymph nodes or a swollen spleen.

Certain factors affect treatment options and prognosis (chance of recovery).

The treatment options may depend on the following:

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

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

  • Whether the hairy cell leukaemia does not grow or grows so slowly it does not need treatment.
  • Whether the hairy cell leukaemia 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 leukaemia are gone). If the leukaemia returns after it has been in remission, retreatment often causes another remission.

Stages of Hairy Cell Leukaemia

There is no standard staging system for hairy cell leukaemia.

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

Untreated hairy cell leukaemia

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

  • Hairy (leukaemia) cells are found in the blood and bone marrow.
  • The number of red blood cells, white blood cells, or platelets may be lower than normal.
  • The spleen may be larger than normal.

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

There are different types of treatment for patients with hairy cell leukaemia.

Different types of treatment are available for patients with hairy cell leukaemia. 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.

Five types of standard treatment are used:

Watchful waiting

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

Chemotherapy

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 leukaemia. These drugs may increase the risk of other types of cancer, especially Hodgkin lymphoma and non-Hodgkin lymphoma. Long-term follow up for second cancers is very important.

Biologic therapy

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 leukaemia.

Surgery

Splenectomy is a surgical procedure to remove the spleen.

Targeted therapy

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 used to treat hairy cell leukaemia.

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 leukaemia.

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. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

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.

Follow-up tests may be needed.

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 Leukaemia

Treatment of hairy cell leukemia may include the following:

  • Chemotherapy.
  • Biologic therapy.
  • Splenectomy.
  • A clinical trial of chemotherapy and targeted therapy with a monoclonal antibody (rituximab).

Treatment Options for Relapsed or Refractory Hairy Cell Leukemia

For information about the treatments listed below, see the Treatment Option Overview section.

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

  • Chemotherapy.
  • Biologic therapy.
  • Targeted therapy with a monoclonal antibody (rituximab).
  • High-dose chemotherapy.
  • A clinical trial of a new biologic therapy.
  • A clinical trial of a new targeted therapy.
  • A clinical trial of chemotherapy and targeted therapy with a monoclonal antibody (rituximab).

Source: National Cancer Institute
Updated: May 18, 2018


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