Acute Lymphocytic 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 (such as acute lymphocytic leukemia) or chronic, depending on whether the abnormal cells are mature or immature. With acute leukemia, the bone marrow cells are unable to properly mature, and they result in immature cells (called blasts). Blasts continue to reproduce and amass at a rapid pace. Without treatment, patients with acute leukemia will die within several months from complications of infection or bleeding .
Acute lymphocytic leukemia (ALL) is the most common type of leukemia in young children under age 10. ALL also affects adults, particularly those aged 65 and older. In 2007, the American Cancer Society estimates that about 5,000 new cases of ALL will be diagnosed 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. Many patients with leukemia 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 Acute Lymphocytic Leukemia possessing the Philadelphia chromosome. 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.
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 Philadelphia chromosome positive ALL. Dr. Goldberg is continuing his research with Gleevec, Sprycel, and other targeted therapies.
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" 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. Recent studies have suggested that bone marrow transplantation should play a larger role in the treatment of most patients with ALL and the efforts to extend transplant options to older patients with this disease are ongoing.
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. Other tissue and cell samples (possibly from the lymph nodes and cerebrospinal fluid) may be taken in order to guide 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.
Radiologic tests used to diagnose leukemia may include a chest X-ray, bone scan, CT scan, MRI, and/or ultrasound.
RISK FACTORS
The cause of Acute Lymphocytic Leukemia remains unclear in most patients. Studies of survivors of the atomic bomb blasts have suggested that radiation may increase the risk of ALL. There are also higher rates of ALL in more developed countries suggesting that exposure to bacterial infections during early childhood may stimulate the immune system and be protective against childhood leukemia. Smoking is directly related to leukemia because cancer-causing substances in tobacco smoke are absorbed through the lungs and then the blood stream. About 20 percent of adult acute leukemia cases are related to smoking tobacco. Environmental risk factors include cancer-causing industrial chemicals, such as benzene, and high-dose radiation exposure. Some studies suggest that electromagnetic fields are a risk factor for acute leukemia in children. Secondary (post-treatment) leukemias can result from chemotherapy and radiation therapy administered for other cancers.
SYMPTOMS
Symptoms of acute leukemia typically appear and worsen quickly. Abnormal cells may collect in the brain or spinal cord and cause headaches, vomiting, confusion, loss of muscle control, and seizures. Some other common symptoms for acute lymphocytic leukemia include:
- fever, chills, and other flu-like symptoms
- frequent infections
- weakness and fatigue (due to anemia)
- loss of appetite and/or weight loss
- 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
The main treatment for acute lymphocytic leukemia (ALL) is chemotherapy, which may be combined with stem cell transplantation, and/or supportive transfusions of blood. Your hematologist/oncologist will decide which treatment or combination of treatments is best for you. 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
An exciting breakthrough in the treatment of ALL is the use of targeted therapies. 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 Philadelphia chromosome positive ALL, which encompase approximately one-third of adult ALL patients.. 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. Sprycel also works by turning off the abnormal protein in cancer cells produced by the Philadelphia chromosome that causes ALL in some.
Chemotherapy
Chemotherapy is used to destroy leukemic cells and to help control symptoms and side effects of the cancer. A combination of medications is often used. Drugs known as growth factors are sometimes given to increase white blood cell counts after chemotherapy and thus reduce the chance of infection. At The Cancer Center many patients are offered a combination of chemotherapy known as hyper-CVAD - MTX/Ara-C. This complete chemotherapy regimen consisting of six different medications requires repeatitive brief hospitalizations administered over four to six months, but most of the care can be delivered in the outpatient Cancer Center. 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).
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. Recent studies have indicated that stem cell transplantation should play a larger role in the treatment of most adult patients with ALL. Early identification of appropriate donors through a co-ordinated effort between the Leukemia Division and our Transplantation Program help smoothly transition patients through the various phases of their therapy.
Transplantation is an important component of Acute Leukemia therapy. Patients at our center are introduced to members of the transplantation team shortly after diagnosis and early identification of appropriate stem cell donors is begun. Following attainment of remission from the initial induction chemotherapy, most patients with ALL will meet formally with one of the transplantation physicians to discuss the role of this therapy in their particular disease. For patients with high risk leukemias (which can be identified by genetic/molecular testing of the leukemia cells at the time of diagnosis) transplantation can be considered as part of the early therapy. By contrast, for patients with lower risk ALL transplantation strategies may be deferred and only used if relapse occurs. A careful multi-divisional approach to leukemia is critical to success and is facilitated at our center by weekly joint meetings between members of the Leukemia and Transplantation Divisions.
Immunosuppressive Therapy
Immunosuppressants are medications that are used to prevent the rejection of donor stem cells after transplantation.
Radiation Therapy
Total body irradiation may be combined with high-dose chemotherapy and stem cell transplantation. Radiation therapy may also be used as a palliative treatment to treat pain due to bone destruction by leukemic cells and/or if enlarged internal organs are pressing against others.
Supportive Treatment
Whole blood transfusions or transfusions of platelets, white blood cells, and red blood cells may be given as supportive treatment.