From: Robina Suwol
Date: 19 Dec 2005
Remote Name: 220.127.116.11
The term leukemia refers to cancers of the white blood cells, which are also
referred to as 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 are free of the disease without it coming back.
Types of Childhood Leukemia
In general, leukemias are classified into acute (rapidly developing) and
chronic (slowly developing) forms. In children, about 98% of leukemias are
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, which are linked to immune defenses, are involved.
Approximately 60% of children with leukemia have ALL, and about 38% have AML. Although slow-growing 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.
Risk for Childhood Leukemia
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.
Children 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 children 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 children have control over the factors that trigger leukemia, although current studies are
investigating the possibility that some environmental factors may predispose a child to 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 of Leukemia
Because infection-fighting white blood cells
are defective in children with leukemia, these children may experience increased
episodes of fevers and infections.
They may also 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 may also 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 may include:
a.. pain in the bones or joints, sometimes causing a limp
b.. swollen lymph nodes (sometimes called swollen glands) in the neck, groin, or elsewhere
c.. an abnormally tired feeling
d.. poor appetite
In about 12% of children with AML
and 6% of children 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.
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 examination, the doctor
will ask you about any concerns and symptoms you have, your past health, your
family's health, any medications you're taking, any allergies you may have, and
other issues. This is called the medical history.
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 also be examined under a microscope to check for certain specific types of abnormal blood cells that are typically seen in patients with leukemia. Blood chemistries will also be checked.
Then, depending on the results of your child's physical exam and preliminary blood tests, your child may need the following:
a.. a bone marrow biopsy and aspiration, in which marrow samples are removed from the body (usually from the back of the hip) for testing
b.. a lymph node biopsy, in which lymph nodes are removed and examined under a microscope to look for abnormal cells
c.. 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 will be done to determine the specific type of
leukemia. In addition to these basic lab tests, cell evaluations are also
generally done, including genetic studies to distinguish between specific types
of leukemia, as well as certain features of the leukemia cells. Children will
receive anesthesia or sedative medications for any painful procedures.
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 children 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 reoccurring. 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 have certain side effects, including hair loss, nausea and vomiting in the short term, and potential
health problems down the line. As your child is treated for leukemia, your child's cancer treatment team will monitor the child 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 children 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 children can be cured - meaning that they are in permanent
remission - of the disease.
Updated and reviewed by: Robin Miller, MD
Date reviewed: July 2005
Originally reviewed by: Steven Dowshen, MD, and Michael Trigg, MD