What exactly is leukemia?

Leukemia is cancer of the bone marrow, the stuff in the center of our bones that makes the three kinds of blood cells. Red blood cells carry oxygen to your body, white blood cells fight infection, and platelets help your blood to clot. Part of the white blood cell is called a lymphocyte. That's where leukemia starts, which is why the most common kind of childhood leukemia is called acute lymphocytic leukemia (ALL). (It's also referred to as acute lymphoblastic leukemia.) "Basically, one of these lymphocytes has gone bad. It then starts to multiply like crazy and eventually takes over the bone marrow," says Castellino. Because leukemia attacks the bone marrow, home base for all blood production, it can affect any or all of your child's blood cell types. That's why many of the symptoms are blood related: Your child may become anemic because he isn't producing enough red blood cells, feverish because he can't effectively use his white blood cells to fight infection, or bruised because his blood isn't producing enough of the platelets he needs for clotting.

About Leukemia
The term leukemia refers to cancers of the white blood cells (also called leukocytes or WBCs). When a child has leukemia, large numbers of abnormal white blood cells are produced in the bone marrow. These abnormal white cells crowd the bone marrow and flood the bloodstream, but they cannot perform their proper role of protecting the body against disease because they are defective.

As leukemia progresses, the cancer interferes with the body's production of other types of blood cells, including red blood cells and platelets. This results in anemia (low numbers of red cells) and bleeding problems, in addition to the increased risk of infection caused by white cell abnormalities. As a group, leukemias account for about 25% of all childhood cancers and affect about 2,200 American young people each year. Luckily, the chances for a cure are very good with leukemia. With treatment, most children with leukemia will be free of the disease without it coming back.

Types of Leukemia
In general, leukemias are classified into acute (rapidly developing) and chronic (slowly developing) forms. In children, about 98% of leukemias are acute. Acute childhood leukemias are also divided into acute lymphocytic leukemia (ALL) and acute myelogenous leukemia (AML), depending on whether specific white blood cells called lymphyocytes (or myelocytes), which are linked to immune defenses, are involved.

Approximately 60% of children with leukemia have ALL, and about 38% have AML. Although slowgrowing chronic myelogenous leukemia (CML) may also be seen in children, it is very rare, accounting for fewer than 50 cases of childhood leukemia each year in the United States.

Causes
The ALL form of the disease most commonly occurs in younger children ages 2 to 8, with a peak incidence at age 4. But it can affect all age groups.

Kids have a 20% to 25% chance of developing ALL or AML if they have an identical twin who was diagnosed with the illness before age 6. In general, nonidentical twins and other siblings of children with leukemia have two to four times the average risk of developing this illness.

Children who have inherited certain genetic problems such as Li-Fraumeni syndrome, Down syndrome, Kleinfelter syndrome,neurofibromatosis, ataxia telangectasia, or Fanconi's anemia have a higher risk of developing leukemia, as do kids who are receiving medical drugs to suppress their immune systems after organ transplants. Children who have received prior radiation or chemotherapy for other types of cancer also have a higher risk for leukemia, usually within the first 8 years after treatment.

In most cases, neither parents nor kids have control over the factors that trigger leukemia, although current studies are investigating the possibility that some environmental factors may increase the risk that a child will develop the disease. Most leukemias arise from noninherited mutations (changes) in the genes of growing blood cells. Because these errors occur randomly and unpredictably, there is currently no effective way to prevent most types of leukemia.

To limit the risk of prenatal radiation exposure as a trigger for leukemia (especially ALL), women who are pregnant or who suspect that they might be pregnant should always inform their doctors before undergoing tests or medical procedures that involve radiation (such as X-rays).

Regular checkups can spot early symptoms of leukemia in the relatively rare cases where this cancer is linked to an inherited genetic problem, to prior cancer treatment, or to use of immunosuppressive drugs for organ transplants.

Symptoms
Because their infection-fighting white blood cells are defective, kids with leukemia may experience increased episodes of fevers and infections. They also may become anemic, because leukemia affects the bone marrow's production of oxygen-carrying red blood cells. This makes them appear pale, and they may become abnormally tired and short of breath while playing.

Children with leukemia might bruise and bleed very easily, experience frequent nosebleeds, or bleed for an unusually long time after even a minor cut because leukemia destroys the bone marrow's ability to produce clot-forming platelets.

Other symptoms of leukemia can include:

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pain in the bones or joints, sometimes causing a limp swollen lymph nodes (sometimes called swollen glands) in the neck, groin, or elsewhere an abnormally tired feeling poor appetite

In about 12% of kids with AML and 6% of those with ALL, spread of leukemia to the brain causes headaches, seizures, balance problems, or abnormal vision. If ALL spreads to the lymph nodes inside the chest, the enlarged gland can crowd the trachea (windpipe) and important blood vessels, leading to breathing problems and interference with blood flow to and from the heart.

Diagnosis
Your child's doctor will perform a physical examination to check for signs of infection, anemia, abnormal bleeding, and swollen lymph nodes. The doctor will also feel your child's abdomen to see if there is an enlarged liver or spleen because they can become enlarged with some cancers in children.

In addition to doing a physical exam, the doctor will take a medical history by asking you about symptoms, past health, your family's health history, medications your child is taking, allergies, and other issues.

After this exam, the doctor will order a CBC (complete blood count) to measure the numbers of white cells, red cells, and platelets in your child's blood. A blood smear will be examined under a microscope to check for certain specific types of abnormal blood cells usually seen in patients with leukemia. Blood chemistries will also be checked.

Then, depending on the results of the physical exam and preliminary blood tests, your child may need:

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a bone marrow biopsy and aspiration, in which marrow samples are removed (usually from the back of the hip) for testing a lymph node biopsy, in which lymph nodes are removed and examined under a microscope to look for abnormal cells a lumbar puncture (spinal tap), where a sample of spinal fluid is removed from the lower back and examined for evidence of abnormal cells. This will show whether the leukemia has spread to the central nervous system (brain and spinal cord).

Bone marrow or lymph node samples will be examined and additional testing done to determine the specific type of leukemia. In addition to these basic lab tests, cell evaluations might be done, including genetic studies to distinguish between specific types of leukemia and certain features of the leukemia cells. Kids will receive anesthesia or sedative medications for any painful procedures.

Treatment
Certain features of a child's leukemia, such as age and initial white blood cell count, are used in determining the intensity of treatment needed to achieve the best chance for cure. Although all kids with ALL are treated with chemotherapy, the dosages and drug combinations may differ.

To decrease the chance that leukemia will invade the child's central nervous system, patients receive intrathecal chemotherapy, the administration of cancer-killing drugs into the cerebrospinal fluid around the brain and spinal cord.

Radiation treatments, which use high-energy rays to shrink tumors and keep cancer cells from growing, may be used in addition to intrathecal chemotherapy for certain high-risk patients. Children then require continued close monitoring by a pediatric oncologist, a specialist in childhood cancer.

After treatment begins, the goal is remission of the leukemia (when there is no longer evidence of cancer cells in the body). Once remission has occurred, maintenance chemotherapy is usually used to keep the child in remission. Maintenance chemotherapy is given in cycles over a period of 2 to 3 years to keep the cancer from returning. Leukemia will almost always relapse (reoccur) if this additional chemotherapy isn't given. Sometimes the cancer will return in spite of maintenance chemotherapy, and other forms of chemotherapy will then be necessary.

Sometimes a bone marrow transplant may be necessary in addition to or instead of chemotherapy, depending on the type of leukemia a child has. During a bone marrow transplant, healthy bone marrow is introduced into a child's body.

Intensive leukemia chemotherapy has certain side effects, including hair loss, nausea and vomiting, and increased risk for infection or bleeding in the short term, as well as other potential health problems down the line. As your child is treated for leukemia, the cancer treatment team will watch closely for those side effects.

But with the proper treatment, the outlook for kids who are diagnosed with leukemia is quite good. Some forms of childhood leukemia have a remission rate of up to 90%; all kids then require regular maintenance chemotherapy and other treatment to continue to be cancer-free. Overall cure rates differ depending on the specific features of a child's disease. Most childhood leukemias have very high remission rates. And the majority of kids can be cured (meaning that they are in permanent remission) of the disease.

Pharmacologic Interventions Acute leukemia Different types of leukemia are best treated with different kinds of medicine.

Acute lymphoblastic leukemia (ALL) drugs include prednisone, vincristine, daunorubicin, L-asparaginase or pegaspargase, methotrexate, and cyclophosphamide. Imatinib(Gleevec) is sometimes used to treat ALL. Dasatinib (Sprycel) is a newer drug for treating some ALL that has not improved with other drugs. Acute myelogenous leukemia (AML) drugs include daunorubicin, idarubicin, cytosine arabinoside, and mitoxantrone.10 Gemtuzumab (Mylotarg) may be given to people whose AML has relapsed. It helps your body destroy cancer cells. Acute promyelocytic leukemia (APL) drugs include all-trans-retinoic acid (ATRA) and chemotherapy with arsenic trioxide, idarubicin, or daunorubicin. ATRA helps control the risk of life-threatening bleeding from disseminated intravascular coagulation (DIC). Later treatment can include ATRA with or without methotrexate and 6mercaptopurine. Or if a first round of ATRA and chemotherapy does not work, arsenic trioxide may be used.10 To treat leukemia in the brain or prevent it from spreading to the brain and central nervous system, methotrexate and cytarabine/cytosine arabinoside are injected into the spinal canal. This is called intrathecal chemotherapy.

Supportive treatments during cancer treatment include:

    

Antibiotics and immunoglobulins help to prevent or fight infections. This is important when you do not have enough normal white blood cells to fight infections on your own. Transfusions of red blood cells and platelets. Epoetin and hematopoietic stimulants help your body make new blood cells. Allopurinol to prevent kidney problems and gout. Saline or steroid eyedrops for relief during treatment with cytarabine/cytosine arabinoside.

Chronic leukemia

    

Chemotherapy for chronic leukemia can involve a single drug or a combination of drugs. For example, you may be given a combination of cyclophosphamide, vincristine, andprednisone. Other drug choices include fludarabine, chlorambucil, hydroxyurea (hydroxycarbamide), cytarabine, busulfan, rituximab, and alemtuzumab. Allopurinol may be given to prevent kidney problems and gout. Dasatinib (Sprycel) blocks the growth of cancer cells. It can be used for CML that has not been helped by imatinib or other drugs. Imatinib (Gleevec) blocks the growth of cancer cells. It is often given to people who havechronic myelogenous leukemia (CML). Immune globulin (IG) helps prevent infections. It is sometimes used for people withchronic lymphocytic leukemia (CLL), because CLL weakens the immune system. Interferon alfa helps your immune system fight disease and may keep cancer cells from growing. It is often given to people who have CML.

Medication for nausea and vomiting Nausea and vomiting are common side effects of chemotherapy. These side effects usually are temporary and go away when treatment is stopped. Your doctor will prescribe drugs to help relieve nausea. These may include: Aprepitant (Emend), which is used in combination with ondansetron and dexamethasone as part of a 3-day program.  Dimenhydrinate, such as Dramamine.  Metoclopramide, such as Reglan and Octamide.  Phenothiazines, such as Compazine and Phenergan.  Serotonin antagonists, such as ondansetron (Zofran), granisetron (Kytril), or dolasetron (Anzemet). These drugs work best when they are combined with corticosteroids such as dexamethasone (Hexadrol). Nursing Interventions Preventing infection:  Frequently monitor the client for pneumonia, pharyngitis, esophagitis, perianal cellulitis, urinary tract infection, and cellulitis, which are common in leukemia and which carry significant morbidity and mortality.  Monitor for fever, flushed appearance, chills, tachycardia; appearance of white patches in the mouth; redness, swelling, heat or pain in the eyes, ears, throat, skin, joints, abdomen, rectal and perineal areas; cough, changes in sputum; skin rash.  Check results of granulocyte counts. Concentrations less than 500/mm3 put the patient at serious risk for infection.  Avoid invasive procedures and trauma to skin or mucous membrane to prevent entry of microorganisms.  Use the following rectal precautions to prevent infections: Avoid diarrhea and constipation, which can irritate the rectal mucosa, avoid the use of rectal thermometers, and keep perineal are clean.  Care for the patient in private room with strict handwashing practice.  Encourage and assist patient with personal hygiene, bathing, and oral care.  Obtain cultures and administer antimicrobials promptly as directed. Preventing and Managing bleeding:  Watch for signs of minor bleeding, such as petechiae, ecchymosis, conjunctival hemorrhage, epistaxis, bleeding gums, bleeding at puncture sites, vaginal spotting, heavy menses.  Be alert for signs of serious bleeding, such as headache with change in responsiveness, blurred vision, hemoptysis, hematemesis, melena, hypotension, tachycardia, dizziness.  Test all urine, stool, emesis for gross and occult blood.  Monitor platelet counts daily.

Administer blood components as directed. Keep patient on bed rest during bleeding episodes. Patient Education and Health Maintenance:  Teach signs and symptoms of infection and advise whom to notify.  Encourage adequate nutrition to prevent emaciation from chemotherapy.  Teach avoidance of constipation with increased fluid and fiber, and good perineal care.  Teach bleeding precautions.  Encourage regular dental visits to detect and treat dental infections and disease.