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Alternative Names: Chronic Granulocytic Leukemia, Chronic Myelocytic Leukemia, Chronic Myeloid Leukemia, Chronic Myelogenous Leukemia.
Chronic Myeloid Meukemia (CML) is a type of cancer that starts in the blood-forming cells of the bone marrow and invades the blood. It is usually associated with a chromosome abnormality called the Philadelphia chromosome. In CML, leukemia cells tend to build up in the body over time, but in many cases people don't have any symptoms for at least a few years. In time, the cells can also invade other parts of the body, including the spleen. CML can also change into a fast-growing acute leukemia that invades almost any organ in the body.
Leukemia is different from other types of cancer that start in organs such as the lungs, colon, or breast and then spread to the bone marrow. Cancers that start elsewhere and then spread to the bone marrow are not leukemia.
Incidence; Causes & Development; Risk Factors
About 4,400 new cases of Chronic Myelogenous Leukemia (CML) are diagnosed each year in the United States.
Most cases of chronic myelogenous leukemia occur in adults, but children may develop the disease. Chronic myelogenous leukemia accounts for about 4% of childhood leukemia cases. Childhood cases (under 20 years of age) represent about 2% of all patients who develop chronic myelogenous leukemia. The frequency of the disease increases with age from about one in 1 million children in the first 10 years of life to one in 100,000 people at age 50, to one in 10,000 people at age 80 and above. The disease in children is similar in behavior to that of adults; however, the outcome of stem cell transplantation is better in younger individuals.
CML results from an acquired (not inherited) injury to the DNA of a stem cell in the marrow. This injury is not present at birth. Scientists do not yet understand what produces this change in the DNA in patients with CML. This change in the stem cell's DNA confers a growth and survival advantage on the malignant stem cell. The result of this injury is the uncontrolled growth of white cells leading, if unchecked, to a massive increase in their concentration in the blood. Unlike acute myelogenous leukemia (AML), chronic myelogenous leukemia permits the development of mature white blood cells that generally can function normally. This important distinction from acute leukemia accounts for the less severe early course of the disease.
Chronic myelogenous leukemia is distinguished from other leukemias by the presence of a genetic abnormality in blood cells, called the Philadelphia chromosome. The changes that result in this chromosome "causing" chronic myelogenous leukemia have been studied intensively. In 1960, two physicians studying chromosomes in cancer cells noticed that a chromosome in CML patients was shorter in length than that of the same chromosome in normal cells. They named this shortened chromosome the Philadelphia chromosome, because the observation was made at the University of Pennsylvania School of Medicine in that city.
The total of 46 chromosomes in normal human cells is composed of 22 pairs of chromosomes numbered 1 to 22 and two sex chromosomes (either an X and Y in males or two Xs in females). The Philadelphia chromosome (No. 22), which is an abnormally short chromosome, is usually referred to as the Ph-chromosome.
Further studies established that two chromosomes, usually chromosome Nos. 9 and 22, were abnormal. Pieces of the chromosomes, which are broken off in the blood cells of patients with chronic myelogenous leukemia, switch with each other. The detached portion of chromosome 9 sticks to the broken end of chromosome 22, and the detached portion of chromosome 22 sticks to the broken end of chromosome 9. This abnormal exchange of parts of chromosomes is called a translocation. This translocation of chromosome pieces occurs only in the stem cell and in the various blood cells derived from that stem cell. The chromosomes of the cells in other tissues are normal.
The cause of the chromosomal breakage in virtually all CML patients is not known. In a small proportion of patients, the cause of the breakage is exposure to very high doses of radiation. This effect has been most carefully studied in the Japanese survivors of the atomic bomb, whose leukemia risk was significantly increased.
A slight increase in risk also occurs in some individuals treated with high dose radiotherapy for other cancers, such as lymphoma. Exposures to diagnostic dental or medical X-rays have not been associated with a heightened risk of chronic myelogenous leukemia.
Signs & Symptoms
The onset of chronic myelogenous leukemia is associated with symptoms that usually develop gradually. Most patients feel a loss of well-being. They tire more easily and may feel short of breath when physically active. They may have a pale complexion from anemia. Discomfort on the left side of the abdomen from an enlarged spleen is a frequent complaint. Patients may experience excessive sweating, weight loss and inability to tolerate warm temperatures.
Diagnosis & Tests
Increasingly, the disease is discovered during the course of a "routine" medical examination. Since the disease worsens over weeks or months, most patients would have symptoms develop soon after such a medical examination in any case.
To diagnose the disease, the blood and, in most cases, the marrow cells must be examined. The white cell count invariably increases, often to very high levels. Examination of the stained (dyed) blood cells with a light microscope shows a characteristic pattern of white cells: a small proportion of very immature cells (leukemic blast cells), and a larger proportion of maturing and fully-matured white cells (myelocytes and neutrophils).
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Signs, symptoms & indicators of Chronic Myelogenous Leukemia (CML):
Conditions that suggest Chronic Myelogenous Leukemia (CML):
Chronic Myelogenous Leukemia (CML) suggests the following may be present:
Recommendations and treatments for Chronic Myelogenous Leukemia (CML):
KEY | | Weak or unproven link |  | | Strong or generally accepted link |  | | Proven definite or direct link |  | | Likely to help |
GLOSSARY
Abdomen (Abdominal)
That part of the body between the chest and the hips that contains the stomach, intestines, liver, bladder, pancreas and other organs.
Acute
An illness or symptom of sudden onset, which generally has a short duration.
Anemia (Anaemia, Anemias)
A condition resulting from an unusually low number of red blood cells or too little hemoglobin in the red blood cells. The most common type is iron-deficiency anemia in which the red blood cells are reduced in size and number, and hemoglobin levels are low. Clinical symptoms include shortness of breath, lethargy and heart palpitations.
Bone Marrow
The soft inner part of the bones, where new blood cells are made.
Cancer
Refers to the various types of malignant neoplasms that contain cells growing out of control and invading adjacent tissues, which may metastasize to distant tissues.
Chronic (Chronicity)
Usually referring to chronic illness: Illness extending over a long period of time.
Colon (Colonic)
The part of the large intestine that extends to the rectum. The colon takes the contents of the small intestine, moving them to the rectum by contracting.
DNA
Deoxyribonucleic acid, the large molecule that is the main carrier of genetic information in cells. DNA is found mainly in the chromosomes of cells.
Leukemia
Cancer of the lymph glands and bone marrow resulting in overproduction of white blood cells (related to Hodgkin's disease).
Lung (Lungs, Pulmonary)
Organ of the body, located in the chest cavity which is designed to bring oxygen from the air into the blood stream, while also expelling carbon dioxide and other waste gases out of the body. Pulmonary: Related to the lungs.
Lymphoma (Lymphomas)
Any tumor of the lymphatic tissues.
Malignant (Cancerous)
Dangerous. Mainly used to describe a cancerous growth -- when used this way, it means the growth is cancerous and predisposed to spreading.
Neutrophils (Neutrophil)
Another name for polymorphonuclear leukocytes, the most common type of blood-carried white blood cell, and the first mobile resistance cell to come to the rescue in injury.
Radiation Therapy (Radiotherapy)
The use of high-energy rays to kill cancer cells. Depending on the stage of the disease, treatment with radiation may be given alone or with chemotherapy. Radiation therapy is local therapy; it affects cancer cells only in the treated area. External radiation does not cause the body to become radioactive. Most often, treatment is given on an outpatient basis in a hospital or clinic.
Spleen (Splenectomy)
The largest organ of the lymphatic system in the body, located between the stomach and the diaphragm (the muscle below the lungs which provides the motion for breathing). It destroys old red blood cells, produces lymphocytes and plasma cells, and stores blood. It also has other as yet unknown functions. A splenectomy is the surgical removal of the spleen.
White Blood Cell (WBC, White Blood Cells)
A blood cell that does not contain hemoglobin: a blood corpuscle responsible for maintaining the body's immune surveillance system against invasion by foreign substances such as viruses or bacteria. White cells become specifically programmed against foreign invaders and work to inactivate and rid the body of a foreign substance. White blood cells are composed primarily of neutrophils, monocytes and lymphocytes. Lymphocytes are either T-cells or B-cells. T-cells (CD3 cells) are divided into T-helper (CD4 cells) and T-suppressor/cytotoxic (CD8 cells) cells.
X-rays (X-ray)
High-energy radiation used to take pictures of areas inside the body.
Last updated: Nov 14, 2009
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