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Chronic myelogenous leukemia (CML) is a slowly progressing blood and bone marrow disease that usually occurs during or after middle age, and rarely occurs in children.
Normally, the body produces bone marrow stem cells (immature cells) that develop into mature blood cells.
There are 3 types of mature blood cells:
In CML, the body tells too many bone marrow stem cells to develop into a type of white blood cell called granulocytes. Some of these bone marrow stem cells never become mature white blood cells. These are called blasts. Over time, the granulocytes and blasts crowd out the red blood cells and platelets in the bone marrow.
Possible signs of chronic myelogenous leukemia include tiredness, night sweats, and fever.
SYMPTOMS
These and other symptoms may be caused by CML. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
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 develop into white blood cells (granulocytes or blasts).
The Philadelphia chromosome is not passed from parent to child.
Tests that examine the blood and bone marrow are used to detect (find) and diagnose chronic myelogenous leukemia.
The following tests and procedures may be used:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
STAGES
After chronic myelogenous leukemia 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 leukemia (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 following tests and procedures may be used to find out the phase:
Chronic myelogenous leukemia 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 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 Leukemia
In relapsed CML, the number of blast cells increases after a remission.
Treatment Option Overview
Six types of standard treatment are used:
Tyrosine kinase inhibitor therapy
A drug called imatinib mesylate is used as initial treatment for certain types of chronic myelogenous leukemia in newly diagnosed patients. It blocks an enzyme called tyrosine kinase that causes stem cells to develop into more white blood cells (granulocytes or blasts) than the body needs. Another tyrosine kinase inhibitor called dasatinib is used to treat patients with certain types of CML that have progressed, and is being studied as an initial treatment.
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 spinal column, 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.
Chronic Phase Chronic Myelogenous Leukemia
Treatment of chronic phase chronic myelogenous leukemia may include the following:
Accelerated Phase Chronic Myelogenous Leukemia
Treatment of accelerated phase chronic myelogenous leukemia may include the following:
Information about ongoing clinical trials is available from the NCI Web site.
Blastic Phase Chronic Myelogenous Leukemia
Treatment of blastic phase chronic myelogenous leukemia may include the following:
Treatment of relapsed chronic myelogenous leukemia may include the following: