By Myrrh Hector, About.com
About.com Health's Disease and Condition content is reviewed by V.K. Gadi, MD
What is Leukemia?
Leukemia is a group of cancers that develop when the DNA of an immature white blood cell (or less commonly, an immature red blood cell) is damaged in some way. This damage causes the red or white blood cell to grow and divide out of control, and the abnormal cell is then circulated along with healthy blood cells to the rest of the body.
Normal Blood and Bone MarrowJust like the liver or skin, the bone marrow is an organ. The bone marrow functions to produce blood cells. One part of the bone marrow produces red blood cells and another part produces lymphocytes (white blood cells that are part of the immune system). After they mature, the red blood cells, white blood cells, and platelets formed in the bone marrow are “picked up” by blood that flows through the bone marrow and out to the bloodstream. Blood cells of all types are suspended in plasma to make up the blood that flows throughout the body, and each cell has its own function. Red blood cells bring oxygen to the muscles and organs of the body. Platelets clump together (clot) to slow bleeding from cuts or bruises, and all types of white cells work to fight infections.
Types of Leukemia
Leukemia is divided into four groups according to the type of cell affected and its maturity.
The four main types of leukemia are:
- Acute Lymphocytic Leukemia. Acute lymphocytic leukemia (ALL) is a rapidly progressing leukemia involving lymphocytes, a type of white blood cell.
- Chronic Lymphocytic Leukemia. This type of leukemia also involves the lymphocytes, but progresses much more slowly than ALL.
- Acute Myelogenous Leukemia. Acute myelogenous leukemia (AML) involves myeloid stem cells, and progresses quickly, requiring immediate treatment.
- Chronic Myelogenous Leukemia.This type of leukemia also involves myeloid stem cells, but like CLL, progresses more slowly than the acute forms of leukemia.
Risk Factors for Leukemia
Doctors and researchers are not sure what exactly causes leukemia, but they have identified a few factors that can increase the risk of developing leukemia. Having one or more of these risk factors does not mean that leukemia is inevitable; these are just some common factors that experts have noticed among patients diagnosed with leukemia. These include:
- Exposure to dangerous chemicals such as benzene or formaldehyde. Workers in the chemical industry may be exposed to these chemicals as part of their daily duties, which places them at increased risk for leukemia.
- Exposure to high levels of radiation. Exposure can come from radiation treatment for another cancer, or being near the explosion of a nuclear bomb or the site of a nuclear power plant leak or explosion. Lower levels of radiation, like those used in x-rays have not been shown to increase the risk of developing leukemia.
- Prior chemotherapy. Chemotherapy for other cancers, especially with a type of drug known as an alkylating agent, can increase the risk of developing leukemia.
- Having Downs Syndrome. The presence of this disorder (or less commonly other disorders) involving abnormalities of the chromosomes can increase the risk of leukemia.
- Having myelodysplastic syndrome. Myelodysplastic syndromes (MDS) are a group of diseases in which the bone marrow does not make enough blood cells, or makes blood cells that die quickly once they are in the bloodstream. MDS can be deadly in its own right, even if it does not progress into leukemia. Some consider MDS to be a kind of "pre-leukemia."
When the first symptoms of leukemia appear, the person affected may not realize that anything out of the ordinary is happening. The symptoms can be very vague, and it's easy to blame things like excessive stress or even the flu for some. Common symptoms of leukemia include:
- Headache. Leukemia can cause frequent headaches when large numbers of lymphocytes (white cells) collect in the brain and spinal cord.
- Weakness or tired. The fatigue and feelings of weakness are caused by a shortage of healthy red blood cells to supply the muscles with oxygen. This shortage of red blood cells is called anemia.
- Pain in the bones or joints. Joint and bone pain is caused by abnormal white blood cells collecting under a bone's surface or in a joint.
Leukemia also has some distinctive signs. While symptoms are subjective feelings like pain, tiredness, or weakness that can only be felt by the person experiencing them, signs are indicators of a health problem that can be observed or measured by an outside person such as a doctor or nurse, things like laboratory results, changes in weight, or paleness. Signs of leukemia include:
- Abdominal or lymph-node swelling. Large numbers of abnormal white blood cells can collect in the spleen, causing abdominal swelling, or in the lymph nodes causing them to swell. Swollen lymph nodes can be felt from the outside in the armpits and the neck, but lymph nodes that are located deeper in the body can only be found with an MRI or a CT scan. Abdominal swelling can also be felt externally, although the source of the swelling must be confirmed with a scan.
- Abnormal bruising or bleeding. This sign can present itself in different ways, including frequent nosebleeds, bleeding of the gums (especially after brushing the teeth), or small red spots on the skin (called petechiae) that result from bleeding in tiny blood vessels underneath the skin. This tendency to bruise or bleed easily is caused by a shortage of platelets, the blood cells that clump together to slow or stop bleeding in the body. A shortage of platelets in the blood is called thrombocytopenia.
- Frequent infections. Leukemia can make the body extremely vulnerable to infection, which may seem confusing because of all the discussion of increased numbers of white blood cells. Unfortunately, the abnormal white blood cells that are produced do not have the ability to effectively fight infection, and there are not usually enough healthy white blood cells to keep the immune system strong.
- Fevers. Fevers are one response of the immune system to infections, and are caused by the release of chemicals that tell the brain to raise the body's temperature. When a person has leukemia, these chemicals may be released excessively, causing frequent or persistent fevers (although some may be caused by infections).
- Weight loss. Weight loss in leukemia patients is caused by a variety of factors. The release of certain chemicals caused by leukemia, the toll on the body of frequent infections, and other factors work together to cause weight loss.
- Abnormal blood test results. A complete blood count (a blood test that examines all three types of blood cells) will measure the number of white blood cells, red blood cells, and platelets. A person with leukemia may have much higher than normal levels of white cells, and possibly lowered counts of red blood cells and platelets.
Even if more than one of these signs or symptoms is present, it does not necessarily mean that a person has leukemia. All of these signs and symptoms can point to conditions other than leukemia. So it's important to talk to a doctor about any unusual or troublesome health issues. Leukemia isn't diagnosed based on the presence of one specific sign or symptom, but on a pattern of signs, symptoms, and test results that have been interpreted by a doctor who is qualified to make the diagnosis.
How Leukemia is Diagnosed
Leukemia can be diagnosed with a variety of tests, and understanding what each test is and what it looks for can make the tests a little less confusing. Remember to let friends and family be a source of support during the stressful time of testing and diagnosis, whether it is you who are the patient or your child. While some tests are more definitive than others, usually a doctor uses the results from a variety of exams, lab results, and scans to diagnose the specific type of leukemia, how advanced it is, and what type of treatment is recommended. Some important questions to ask before any test are:
- What will this test tell us?
- How is this test done?
- When will we get the results?
- What is the next step after we get the results of this test?
An exam is much like any doctor's appointment; the doctor will take a detailed medical history and thoroughly examine the whole body. In addition to the standard check of vital signs and breath sounds, the doctor will examine the lymph nodes in the neck and armpits to look for swelling caused by large amounts of white blood cells gathered in the lymph nodes. The doctor will also press on the abdomen (stomach area), noting any tenderness or swelling which would suggest that a large concentration of leukemic white blood cells is enlarging the spleen. A physical exam doesn't usually cause any any side effects, except for the normal worry about a possible leukemia diagnosis.
Blood TestsBlood tests are chemical tests done on a sample of blood in a laboratory. A sample of blood is taken from a patient's vein with a needle and collected in a vial, then sent to a laboratory to be analyzed. The most common blood test for leukemia is the complete blood count, or CBC. A CBC counts the number of white blood cells, red blood cells, and platelets in a blood sample. A sample of blood with high numbers of white blood cells and low levels of red blood cells or platelets can indicate leukemia, and abnormal liver and kidney function tests will tell the doctor if leukemia has affected those organs.One very important test that allows doctors to look specifically for blasts in a blood sample is flow cytometry. Flow cytometry also allows doctors to examine the physical characteristics of the blasts, allowing for more precise diagnosis. Blood draws should only be felt as a quick pinch, so don't be afraid to ask for someone else if the person drawing the sample is having trouble finding a vein.
Bone Marrow Biopsy A biopsy takes a small sample of bone marrow tissue from the body. A bone marrow biopsy or aspiration is done in in the doctor's office. The doctor asks the patient to lie on his or her side and nurses clean the biopsy site with iodine or alcohol. After the site is cleaned, the biopsy site is numbed with a local anesthetic. The doctor uses either a very fine needle to draw out only bone marrow tissue, or a thicker needle to take a small sample of bone and bone marrow. The bone marrow sample is examined under a microscope to check for leukemia cells. This procedure should be relatively painless, as a local anesthetic such as lidocaine will be used, and the inside of the bone has no nerve endings. Patients may request light sedation and narcotics during the procedure. There may be some soreness after the procedure, so it's important to take it easy and follow the doctor's instructions for taking care of the biopsy site. CytogeneticsCytogenetics is a newer type of testing for leukemia, and it looks at the chromosomes in individual cells. Cytogenetic testing uses a sample taken from a blood draw or a bone marrow or lymph node biopsy. The sample's chromosomes are microscopically examined for abnormalities that indicate damage to the cells' DNA.
Spinal TapsA spinal tap involves a doctor taking a sample of cerebrospinal fluid. The samples is usually taken from the lower back (the lumbar area). Spinal taps take place in a procedure room in a doctor's office, and the patient is asked to lie on his or her side. The doctor then cleans the site of the puncture and injects a local anesthetic to numb the area. A special needle called a spinal needle is inserted into the space between vertebrae, and cerebrospinal fluid is drawn through the needle and into a collection tube for analysis. The fluid sample is then checked for leukemia cells and other abnormalities. A spinal tap can cause a severe headache that lasts a long time if the patient moves around too quickly after the procedure. There may also be some small discomfort at the site of the puncture, and as always, it's important to follow any instructions given by the doctor after the spinal tap.
Scans and X-raysMRIs (Magnetic Resonance Imaging), CT (Computerized Axial Tomography) scans, and x-rays are all technologies that use various machines to take pictures of bones, organs, and tissues inside the body. In the case of CT scans and MRIs, the technician has the patient lie down on a table that moves into a tube. CTs and MRIs build detailed 3-D pictures that can be examined by the doctor from any angle. In an x-ray, a patient is positioned so that two-dimensional pictures can be taken of the body. These images are interpreted by a radiologist and the images and a written report are sent back to the doctor. Scans and x-rays allow doctors to visually pinpoint problem areas within the body, and locate areas of infection.
Treating LeukemiaEach type of leukemia has its own typical course of treatment, but there are only a few different types of treatment for leukemia. Explore the way each treatment works, how it is given, and what some of the most common side effects are.
ChemotherapyHow Chemotherapy Works: The body naturally replaces normal cells only when cells die, but leukemic cells will divide constantly, which causes a build-up of cancer cells in the body. Chemotherapy drugs kill all rapidly dividing cells in the body, which is both the reason that chemotherapy works and the reason for most of its side effects. The ultimate goal of chemotherapy is to eliminate any visible signs of leukemia cells in the body; this elimination of any detectable signs of cancer is called remission.
How Chemotherapy is Given: Drugs can be given by mouth (orally) in the form of a pill, by injection into a muscle, or by IV directly into a vein. Instead of putting in a new IV for each chemotherapy session, many patients have a long-term IV catheter inserted, more commonly known as a port. Putting the port in is a minor surgical procedure, and it saves the person receiving treatment from a lot of extra needle sticks. Drawing blood for lab work can usually be done through the port as well.
The combination of drugs used will be different for each patient, but the process of chemotherapy is generally the same. Most leukemias are treated initially with inpatient chemotherapy. This is required because of the intensity of the chemotherapy and because hospital staff can administer blood products and antibiotics to inpatients as needed to keep blood counts higher and ward off infections. Chemotherapy is only halted in an inpatient setting if it is not working. Subsequent cycles of maintenance chemotherapy may be done in an outpatient setting after remission has been acheived.
Common Chemotherapy Side Effects: When people think of chemotherapy, they often think of guaranteed hair loss, endless nausea and vomiting, and lots of suffering and discomfort. However, some patients are able to get through chemotherapy without a lot of side effects, while others have a more difficult time. While side effects can be difficult, the benefits of timely and appropriate treatment will usually outweigh the discomfort, though it may not seem like it at the time! Chemotherapy drugs can cause constipation and diarrhea or nausea and vomiting. Too much of these side effects can cause dehydration or weight loss. In the hospital, weight and body functions are monitored closely and side effects treated as they appear, but it's always helpful to be open and honest about how you're feeling, especially when you are in the hospital.
Hair loss is one of the more well-known effects of chemotherapy, and can be one of the most difficult for male and female leukemia sufferers alike. Fortunately, wigs are much more natural looking than they used to be, and free or low-cost wigs can be obtained through wig banks at your cancer treatment center or in your community. Ask your healthcare team for information, resources, and assistance in dealing with treatment-related hair loss.
Radiation TherapyHow it works: External radiation therapy uses highly complex equipment to deliver an appropriate dose of ionizing radiation to specific places in the body to damage the DNA and ultimately kill cancer cells. Normal cells that are near the site being treated may also be damaged by the radiation, but are usually able to recover and continue functioning. Radiation is specifically used in people with leukemia to target leukemic cells in spots that it's difficult to reach with chemotherapy, like the spaces around the brain and spinal cord.
How it's given: Radiation can be given in a variety of ways, but patients usually have fairly similar experiences. The first visit for radiation therapy is longer and the radiation therapy team uses this visit to specifically identify the site to be irradiated (given radiation). Molds and positioners may also be made during this first visit to help the patient hold still in a certain position. After extensive planning on where and how the radiation dose will be delivered, the patient is positioned on a table in the room with the equipment, and technicians operate the equipment from outside the room. Actual radiation therapy sessions usually last only a few minutes, with extra time if a meeting with the radiation oncologist (a doctor specializing in the treatment of cancer with radiation) is needed.
Common Side Effects: Radiation therapy does have some side effects which may or may not be experienced during or after treatment. The most common side effects noticed are very tired feelings (fatigue) or changes in the skin, including dryness, burning, itching, or peeling. Creams or lotions may be prescribed to treat or lessen these effects, but it's important to follow the radiation therapy team's instructions on caring for the skin during and after treatment. Because leukemia patients often undergo whole brain irradiation, hair loss is a possibility as well. There may be other side effects specific to the site that's being treated, and these will be discussed with the person receiving treatment and their caregiver before treatment begins.Stem Cell Transplantation
How Stem Cell Transplantation works: Stem cell transplantation can be done using either the patient's own cells during a disease remission or cells harvested from a healthy tissue-matched donor, with the latter being done much more often as studies have proved transplants using donor stem cells to be more effective. Transplants are done to replace cells lost to intensive chemotherapy or radiation, and are also valuable in patients receiving cells from a donor because the donor cells will recognize and attack the patient's cells, destroying leukemic cells and asserting themselves as the dominant (and healthy) cells in the patient's bone marrow. This graft versus leukemia effect eliminates leukemia cells that may "hide" from chemotherapy or radiation, and helps decrease the chances of relapse.
How Stem Cell Transplantation is done: The patient receives a course of intense chemotherapy or radiation therapy. Because the plan is to restore bone marrow cells and function with the transplant, higher doses than could normally be given are administered. Stem cells are harvested from the donor, either from the blood or directly from the bone marrow. The stem cells are then given to the leukemia patient through a catheter that is implanted into a vein (called a central line). Until the patient's bone marrow or blood has incorporated the donor cells, the stem cell recipient is very vulnerable to infections and must often stay in the hospital for an extended period of time.
Common side effects: Most of the side effects of the procedure come from the intense chemotherapy or radiation given before the transplant, and may be similar or somewhat more intense to the side effects normally associated with chemotherapy or radiation. The conditioning therapy and the transplant itself both leave the patient without an effective immune system for a short period of time, and this leaves the patient very vulnerable to infection and disease. Transplantation of donor stem cells can also cause an effect called "graft-versus-host." As explained before, this effect can be beneficial, but if it becomes extreme it can develop into a serious condition called graft-versus-host disease. Medications are given before and after the donor cells are infused to reduce the chances of developing the condition.
Other TreatmentsIn addition to conventional chemotherapy and radiation, patients can also be treated with monoclonal antibodies attached to radioactive compounds. This type of treatment is called "radioimmunotherapy."
Leukemia patients may also choose to receive treatment as part of a clinical trial. All patients involved in clinical trials will be given at least the standard treatment, unless they are enrolled in a preliminary clinical trial. Before participating, patients will be told all possible risks.
Whatever treatment is chosen, patients and families should understand the potential risks and benefits. Treatment can't be done without the consent of a patient or their parent or guardian, so it's important to understand the recommended treatment before consent is given.
Many people who suffer from leukemia are able to go on and live full and healthy lives after treatment. They have found ways to integrate leukemia into their life's experiences, healthy methods of emotional expression, and the right follow-up treatment to keep their health the best it can be. Leukemia is not a death sentence, but it does have many challenges, each sometimes requiring creative problem-solving to overcome.
Physical: Depending on the type of leukemia, signs and symptoms may affect the quality of everyday life. It's difficult to cope with everyday routines when fatigue is overwhelming, or when constant fevers and infections force the person with leukemia to stay home from school or work. Nausea from chemotherapy may affect a patient's diet and ability to maintain good nutrition, causing weight loss. It's important to ask doctors and nurses how to deal with bothersome side effects like these. Being honest about symptoms can help doctors to address them, and they can do their best to help those with leukemia feel better each day.
Emotional: Being diagnosed with leukemia can arouse fears and worries about the future, and even after remission fears of relapse or anxieties about the need for future treatment can interfere with daily living. Some ways to deal with the emotions of leukemia can include: building a support system of family and friends, writing down thoughts and feelings in a journal, or seeking professional help from a counselor or therapist. Seeking professional counseling does not mean that a person is crazy or weak; it's a way to get an impartial perspective on feelings and events that are often overwhleming.
Financial: The financial burdens of leukemia treatments can last long after remission. Chemotherapy is expensive and treatment often interferes with a patient's ability to work, or the parents' ability to work if the patient is a child. If the leukemia patient has insurance, that may help cover some of the costs, but often the patient is left with high copays or fees to pay out of his own pocket. If this happens, it's important to talk with the billing department of the hospital or doctor's office about the difficulty or to seek out other sources of financial assistance.
Intellectual : A child who is diagnosed with leukemia is often unable to attend school for long periods of time due to illness or a weakened immune system, interrupting the normal course of that child's education. Also, chemotherapy can have cognitive effects on both children and adults, causing forgetfulness or difficulty learning. Children and adults need to be aware of these possible effects so they can develop strategies to overcome them and continue with education and everyday living.
Sexual : Chemotherapy can affect sexual desire and produce side effects that can inhibit adult patients' ability to be sexual. Certain treatments for leukemia can also affect a child's ability to go through puberty or conceive and bear children. It's important to ask doctors and nurses about the sexual and reproductive side effects of treatment for leukemia and what can be done to alleviate those effects before beginning a treatment.
Source:
http://leukemia.about.com/"Understanding Blood Counts." 25 Oct 2004. Leukemia and Lymphoma Society. 20 Feb 2008.
"How Is Childhood Leukemia Diagnosed?" 19 Aug 2007. American Cancer Society. 20 Feb 2008.
"What You Need to Know About Leukemia." National Cancer Institute. 31 Mar 2003. National Institute of Health. 13 February 2008. 20 Feb 2008."Blood and Marrow Stem Cell Transplantation." 15 Feb 2008. Leukemia and Lymphoma Society. 2 Mar 2008.
"Radiation Therapy for Cancer: Questions and Answers." 25 Aug 2004. National Cancer Institute. 2 Mar 2008.
"Understanding Drug Therapy and Managing Side Effects." 20 Feb 2007. Leukemia and Lymphoma Society. 2 Mar 2008."Coping." 19 January 2006. Leukemia and Lymphoma Society. 2 March 2008.
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