Patient Resources

Chronic Myeloid (Myelogenous) Leukemia

 

 

OVERVIEW

Leukemia is a cancer that begins in the hematopoietic (blood-forming) cells of the bone marrow, the soft, spongy inner part of such bones as the skull, pelvis, and backbones. The hematopoietic cells produce three types of blood cells: red, white, and platelets. Red cells carry oxygen to the muscles and provide the body with energy. Platelets are the clotting cells that prevent bleeding. White blood cells are the cells of the immune system. Leukemia occurs when abnormal blood cells reproduce and crowd out normal cells in the bone marrow. Most leukemias are cancers of the white blood cells. Abnormal leukemic cells can spread the cancer to other parts of the body including the lymph nodes, liver, spleen, skin, and central nervous system. (When cancer starts elsewhere and spreads to the bone marrow, it is not leukemia.)

Leukemia can be acute or chronic (such as chronic myeloid leukemia), depending on whether the abnormal cells are mature or immature. In chronic leukemia, bone marrow cells mature but they do not function properly and do not fight infections as would normal white blood cells. Because chronic leukemic cells can live much longer than normal white blood cells, chronic leukemia is a slower-forming disease that usually worsens gradually.

Chronic myeloid leukemia (CML) forms in the myeloid cells of the bone marrow. It occurs mainly in adults ages 40 to 50. A very small number of children also develop CML. The Leukemia and Lymphoma Society estimates that 5,000 people are diagnosed with CML each year in the United States.

At The Cancer Center, our chief of the Division of Leukemia, Stuart L. Goldberg, M.D., is one of the nation's foremost authorities and researchers of all types of leukemia. He also serves as the medical advisor to the Northern NJ Chapter of the Leukemia and Lymphoma Society. He leads an active leukemia research team that is conducting about 30 clinical trials for new treatments for leukemia. Most patients with CML at The Cancer Center are enrolled in a clinical trial. National Cancer Institute research groups, pharmaceutical companies, and independent research agencies frequently turn to Dr. Goldberg to collaborate on clinical research studies because of his successful accruals (enrollments) and his ability to attract new patients to The Cancer Center. Dr. Goldberg and his team were part of an elite group of researchers who in 2001 were instrumental to developing and bringing the breakthrough chemotherapeutic drug Gleevec to patients with CML. The Cancer Center was the only facility in New Jersey that participated in the nationwide clinical trial for Gleevec that resulted in its approval by the Food and Drug Administration. Gleevec opened up a whole new avenue for the treatment of CML, especially for elderly or vulnerable patients, who could not undergo the rigors of stem cell transplantation to treat their leukemia.

At Leukemia Division at Hackensack University Medical Center has continued its studies with Gleevec in CML exploring the appropriate dosing of this breakthrough medicine. Our center has pioneered high-dose Gleevec trials, becoming lead investigators on the US Right Trial which was presented at the 2006 American Society of Hematology Meetings. The success of this study which demonstrated that high-dose Gleevec helped more patients obtain remission during the first year of treatment, led to the International TOPS randomized trial. Again Hackensack University Medical Center has led the way, becoming one of the largest participants in this trial in the world.

Dr. Goldberg and his research team were also part of an elite group of international researchers from 20 countries who evaluated Sprycel, a second-generation oral chemotherapeutic drug that was approved by the FDA in 2006 for patients with CML. The Cancer Center was one of the top three institutions with the highest number of patients enrolled in the study. Sprycel is used to treat patients whose leukemia is resistant to Gleevec or who suffer side effects from the drug. Dr. Goldberg is continuing his research with Gleevec, Sprycel, and other targeted therapies.

Although Gleevec, Sprycel, and Tasigna (another targeted agent studied at Hackensack University Medical Center) have shown remarkable success, unfortunately not all patients respond to treatment. Dr Goldberg and his team are now embarking on several new strategies to control CML including studies of histone deacytelase inhibition and auroa kinase inhibition.

A hematologist/oncologist, Dr. Goldberg also specializes in the use of stem cell transplantation to treat patients with leukemia. He and his colleagues at The Cancer Center's Adult Blood and Marrow Stem Cell Transplantation Program pioneered the use of reduced-intensity "mini&quot: transplants for older patients and those who are too frail to undergo the rigors of a standard stem cell transplant. Our stem cell transplantation program is one of the nation's 10 largest; each year more than 200 stem cell transplants are performed here. The success of the targeted therapies has resulted in a lesser need for transplantation in CML (once the primary treatment).

A crucial component to the accurate diagnosis of leukemia is The Cancer Center's Special Diagnostic Immunology Laboratory, one of only several sites in New Jersey where comprehensive tests are available to detect cancer at the molecular level and to stage and classify it.

 

DIAGNOSTIC SERVICES

A diagnosis of leukemia is made after several tests have been completed. Samples of cells are taken from a patient's blood and bone marrow. A crucial component to the accurate diagnosis of leukemia is The Cancer Center's Special Diagnostic Immunology Laboratory, one of only several sites in New Jersey where comprehensive tests are available to detect cancer at the molecular level and to stage and classify it.

 

RISK FACTORS

The cause of CML remains unknown. However, studies of survivors of the atomic bomb blasts have suggested that exposure to high dose radiation may increase the risk of developing CML.

 

SYMPTOMS

Symptoms of chronic leukemia emerge slowly and, when they do appear, they are generally mild at first then worsen gradually. About one out of five persons with chronic leukemia has no symptoms at the time that it is found. Some common symptoms for chronic myeloid leukemia include:

  • fever, chills, and other flu-like symptoms
  • frequent infections
  • weakness and fatigue (due to anemia)
  • loss of appetite and/or weight loss
  • .pain in the left abdomen
  • swollen or tender lymph nodes, liver, or spleen
  • easy bruising or bleeding
  • tiny red spots (called petechiae) under the skin
  • swollen or bleeding gums
  • sweating, especially at night
  • bone or joint pain

 

TREATMENT SERVICES

There are several treatment methods for chronic myeloid leukemia. These include revolutionary targeted therapies, stem cell transplantation combined with high-dose chemotherapy and total body irradiation, radiation therapy, interferon-alpha, supportive therapy, and very rarely surgery. Your hematologist/oncologist will decide which treatment or combination of treatments is best for you based on the stage of the disease, your age, your overall health, and whether the leukemia is causing any symptoms. The decision to receive aggressive treatments can often be quite difficult but may be based on the patient's subtype of leukemia, general health, age, and overall desires. Shortly after diagnosis a comprehensive review of the type of leukemia is performed to determine the aggressiveness of the cancer and to provide prognostic information that may be helpful in chosing a treatment course.

Targeted Therapies

By far, the most exciting breakthrough in the treatment of chronic myeloid leukemia (CML) is Gleevec, an oral chemotherapeutic drug that is taken in pill form daily for the rest of a patient's life. Gleevec is known as a "targeted therapy" It zeroes in on leukemic cells and destroys them, while sparing normal cells. When it was approved by the Food and Drug Administration in 2001, it opened up a whole new avenue of treatment options for patients with CML. Previously many younger, healthier patients underwent stem cell transplantation to treat CML, but patients over age 50 were usually too vulnerable to the rigors of stem cell transplantation. Their treatment options were limited until Gleevec was approved.

For patients whose CML is resistant to Gleevec or who cannot tolerate side effects caused by the medicine, Sprycel is another breakthrough second-generation targeted therapy that may be prescribed. In clinical trials conducted by our chief of the Division of Leukemia, Stuart L. Goldberg, M.D., and other researchers in 20 countries, 80 percent of patients who were unable to take Gleevec because of side effects and 40 percent of those whose CML was resistant to Gleevec were able to take Sprycel successfully. Sprycel works by turning off the abnormal protein in cancer cells produced by the Philadelphia chromosome that causes CML. Sprycel stops the progression of CML into a crisis stage. Sprycel induced remission in the majority of patients Dr. Goldberg and his co-investigators from around the world studied in clinical trials prior to Sprycel's approval by the Food and Drug Administration in 2006. Tasigna (AMN-107) is another targeted therapy that is undergoing extensive evaluation at Hackensack University Medical Center and may become available shortly. Novel agents and further studies with the available agents are being tested at our center.

Stem Cell Transplantation

Stem cell transplantation may be used to restore blood-producing stem cells that have been destroyed by leukemia or cancer treatment. Stem cell transplantation is a highly advanced type of treatment that is available at only several hospitals in New Jersey. Our Adult Blood and Marrow Stem Cell Transplantation Program is one of the nation's 10 largest. Each year more than 200 persons undergo stem cell transplantation here at The Cancer Center. The program is the only one in New Jersey where stem cell transplants using cells from unrelated donors are performed. Our chief of the Division of Leukemia, Stuart L. Goldberg, M.D., and other experts in stem cell transplantation at The Cancer Center are responsible for some of the major advances in the techniques used in stem cell transplantation, including the use of reduced-intensity "mini" transplants to treat older patients and those who are too frail to undergo the rigors of a standard transplant.

Immunosuppressive Therapy

Immunosuppressants are medications that are used to prevent the rejection of donor stem cells after transplantation.

Chemotherapy

Chemotherapy such as hydroxyurea is used to destroy leukemic cells and to help control symptoms and side effects of the cancer. New "targeted therapy" oral chemotherapeutic drugs, such as Gleevec and Sprycel, have largely replaced traditional chemotherapy approaches. Rarely for patients with more advanced disease, new chemotherapy agents such as decitabine may be used. A more complete discussion of the risks and benefits of chemotherapy will be provided during the extensive consultation process with members of the Division of Leukemia. Additional updates on current treatment approaches are available from the Leukemia and Lymphoma Society (www.LLS.org) and the National Cancer Institute (www.cancer.gov).

Interferons

Interferons are a family of substances naturally produced by several types of cells. Daily injections of interferon-alpha may be used to treat CML to reduce the growth and development of leukemic cells and to promote the immune system's attack against leukemic cells.

Radiation Therapy

Radiation therapy is rarely used to treat leukemia except to irradiate the entire body before stem cell transplantation or to treat pain due to bone destruction by leukemic cells and/or if enlarged internal organs are pressing against others.

Surgery

The only type of surgery used to treat leukemia is a splenectomy (removal of the spleen) if the organ is enlarged and pressing against other internal organs and causing pain.

Supportive Treatment

Whole blood transfusions or transfusions of platelets, white blood cells, and red blood cells may be given as supportive treatment.