Chronic myelogenous leukemia

Subject: Chronic myelogenous leukemia Thu Jan 20, 2011 4:20 pm

Chronic myelogenous leukemia
Filed under: Cancer & Chemo
Chronic myelogenous leukemia (CML) is an uncommon type of cancer of the
blood cells. The term "chronic" in chronic myelogenous leukemia
indicates that this cancer tends to progress more slowly than acute
forms of leukemia. The term "myelogenous" (mi-uh-LOJ-uh-nus) in chronic
myelogenous leukemia refers to the type of cells affected by this
cancer.

Chronic myelogenous leukemia can also be called chronic myeloid leukemia
and chronic granulocytic leukemia. Chronic myelogenous leukemia
typically affects older adults and rarely occurs in children, though it
can occur at any age.

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Symptoms

Signs and symptoms of chronic myelogenous leukemia may include:

* Easy bleeding
* Feeling run-down or tired
* Fever
* Frequent infections
* Losing weight without trying
* Loss of appetite
* Pain or fullness below the ribs on the left side
* Pale skin
* Sweating excessively during sleep (night sweats)

When to see a doctor
Chronic myelogenous leukemia doesn't always reveal itself with obvious
signs and symptoms during the early phase. It's possible to live with
chronic myelogenous leukemia for months or years without realizing it.

Because people with chronic myelogenous leukemia tend to respond better
to treatment when it's started early, make an appointment with your
doctor if you have signs or symptoms that bother you.

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Causes

Chronic myelogenous leukemia occurs when something goes awry in the
genes of your blood cells. It's not clear what initially sets off this
process, but doctors have discovered how it progresses into chronic
myelogenous leukemia.

First, an abnormal chromosome develops
Human cells normally contain 23 pairs of chromosomes. These chromosomes
hold the DNA that contains the instructions (genes) that control the
cells in your body. In people with chronic myelogenous leukemia, the
chromosomes in the blood cells swap sections with each other. A section
of chromosome 9 switches places with a section of chromosome 22,
creating an extra-short chromosome 22 and an extra-long chromosome 9.

The extra-short chromosome 22 is called the Philadelphia chromosome,
named for the city where it was discovered. The Philadelphia chromosome
is present in the blood cells of 90 percent of people with chronic
myelogenous leukemia.

Second, the abnormal chromosome creates a new gene
The Philadelphia chromosome creates a new gene. Genes from chromosome 9
combine with genes from chromosome 22 to create a new gene called
BCR-ABL. The BCR-ABL gene contains instructions that tell the abnormal
blood cell to produce too much of a protein called tyrosine kinase.
Tyrosine kinase promotes cancer by allowing certain blood cells to grow
out of control.

Third, the new gene allows too many diseased blood cells
Your blood cells originate in the bone marrow, a spongy material inside
your bones. When your bone marrow functions normally, it produces
immature cells (blood stem cells) in a controlled way. These cells then
mature and specialize into the various types of blood cells that
circulate in your body — red cells, white cells and platelets.

In chronic myelogenous leukemia, this process doesn't work properly. The
tyrosine kinase caused by the BCR-ABL gene causes too many white blood
cells. Most or all of these contain the abnormal Philadelphia
chromosome. The diseased white blood cells don't grow and die like
normal cells. The diseased white blood cells build up in huge numbers,
crowding out healthy blood cells and damaging the bone marrow.

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Risk factors

Factors that increase the risk of chronic myelogenous leukemia:

* Older age
* Being male
* Radiation exposure, such as radiation therapy for certain types of cancer

Family history is not a risk factor
The chromosome mutation that leads to chronic myelogenous leukemia isn't
passed from parents to offspring. This mutation is believed to be
acquired, meaning it develops after birth.

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Complications

Chronic myelogenous leukemia (CML) can cause a variety of complications, including:

* Fatigue. If diseased white blood cells crowd out healthy red blood
cells, anemia may result. Anemia can make you feel tired and worn down.
Treatment for CML also can cause a drop in red blood cells.
* Excess bleeding. Blood cells called platelets help control
bleeding by plugging small leaks in blood vessels and helping your blood
to clot. A shortage of blood platelets (thrombocytopenia) can result in
easy bleeding and bruising, including frequent or severe nosebleeds,
bleeding from the gums, or tiny red dots caused by bleeding into the
skin (petechiae).
* Pain. CML can cause bone pain or joint pain as the bone marrow expands when excess white blood cells build up.
* Enlarged spleen. Some of the extra blood cells produced when you
have CML are stored in the spleen. This can cause the spleen to become
swollen or enlarged. Rarely, the spleen becomes so large that it's at
risk of bursting. More commonly, the swollen spleen takes up space in
your abdomen and makes you feel full even after small meals or causes
pain on the left side of your body below your ribs.
* Stroke or excess clotting. Some people with CML produce too many
platelets. Without treatment, this high platelet count (thrombocytosis)
can cause excessive clotting of your blood, which can lead to stroke.
* Infection. White blood cells help the body fight off infection.
Although people with CML have too many white blood cells, these cells
are often diseased and don't function properly. As a result, they aren't
able to fight infection as well as healthy white cells can. In
addition, treatment can cause your white cell count to drop too low
(neutropenia), also making you vulnerable to infection.
* Death. If CML can't be successfully treated, it ultimately is fatal.

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Preparing for your appointment

Start by making an appointment with your family doctor or a general
practitioner if you have any signs or symptoms that worry you. If blood
tests or other tests and procedures suggest leukemia, your doctor may
refer you to a specialist in the treatment of blood and bone marrow
diseases and conditions (hematologist).

Because appointments can be brief, and because there's often a lot of
ground to cover, it's a good idea to be well prepared. Here's some
information to help you get ready, and what to expect from your doctor.

What you can do

* Be aware of any pre-appointment restrictions. At the time you make
the appointment, be sure to ask if there's anything you need to do in
advance, such as restrict your diet.
* Write down any symptoms you're experiencing, including any that
may seem unrelated to the reason for which you scheduled the
appointment.
* Write down key personal information, including any major stresses or recent life changes.
* Make a list of all medications, as well as any vitamins or supplements, that you're taking.
* Take a family member or friend along. Sometimes it can be
difficult to absorb all the information provided during an appointment.
Someone who accompanies you may remember something that you missed or
forgot.
* Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions
will help you make the most of your time together. List your questions
from most important to least important in case time runs out. For
chronic myelogenous leukemia, some basic questions to ask your doctor
include:

* Can you explain what my test results mean?
* Do you recommend any other tests or procedures?
* What is the phase of my CML?
* What are my treatment options?
* What side effects are likely with each treatment?
* How will treatment affect my daily life?
* Which treatment options do you think are best for me?
* How likely is it that I'll achieve remission with the treatments you recommend?
* How quickly must I make a decision on my treatment?
* Should I get a second opinion from a CML specialist? What will that cost, and will my insurance cover it?
* Are there any brochures or other printed material that I can take with me? What websites do you recommend?

What you can do in the meantime
Avoid anything that makes your symptoms worse. For instance, if you're
feeling fatigued, rest as much as possible. Focus on only the essential
tasks of each day. The nonessential tasks can wait for a later day.

If you're having trouble eating because you feel full very quickly,
choose smaller, more frequent meals. Consider sipping a nutritional
supplement shake. These high-calorie shakes can help you temporarily to
get the calories and nutrition you need if eating is uncomfortable.

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Tests and diagnosis

Tests and procedures used to diagnose chronic myelogenous leukemia include:

* Physical exam. Your doctor will examine you and check such vital
signs as pulse and blood pressure. He or she will also feel your lymph
nodes, spleen and abdomen for abnormalities.
* Blood tests. A complete blood count (CBC) may reveal abnormalities
in your blood cells. Blood chemistry tests to measure organ function
may also reveal abnormalities that can help your doctor make a
diagnosis.
* Bone marrow tests. Bone marrow biopsy and bone marrow aspiration
are used to collect bone marrow samples for laboratory testing. These
tests involve collecting bone marrow from your hipbone.
* Tests to look for the Philadelphia chromosome. Specialized tests,
such as fluorescence in situ hybridization (FISH) analysis and
polymerase chain reaction (PCR) test, analyze blood or bone marrow
samples for the presence of the Philadelphia chromosome or the BCR-ABL
gene.

Phases of chronic myelogenous leukemia
The phase of chronic myelogenous leukemia refers to the aggressiveness
of the disease. Your doctor determines the phase by measuring the
proportion of diseased cells to healthy cells in your blood or bone
marrow. A higher proportion of diseased cells means chronic myelogenous
leukemia is at a more advanced stage.

Phases of chronic myelogenous leukemia include:

* Chronic. The chronic phase is the earliest phase and generally has the best response to treatment.
* Accelerated. The accelerated phase is a transitional phase when the disease becomes more aggressive.
* Blastic. Blastic phase is a severe, aggressive phase that becomes life-threatening.

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Treatments and drugs

The goal of chronic myelogenous leukemia treatment is to eliminate the
blood cells that contain the abnormal BCR-ABL gene that causes the
overabundance of diseased blood cells. For most people, it's not
possible to eliminate all diseased cells, but treatment can help achieve
a long-term remission of the disease.

Targeted drugs
Targeted drugs are designed to attack cancer by focusing on a specific
aspect of cancer cells that allows them to grow and multiply. In chronic
myelogenous leukemia, the target of these drugs is the protein produced
by the BCR-ABL gene — tyrosine kinase. Targeted drugs that block the
action of tyrosine kinase include:

* Imatinib (Gleevec)
* Dasatinib (Sprycel)
* Nilotinib (Tasigna)

Targeted drugs are the initial treatment for most people diagnosed with
chronic myelogenous leukemia. If the disease doesn't respond or becomes
resistant to the first targeted drug, doctors may consider other
targeted drugs or other treatments. Side effects of these targeted drugs
include swelling or puffiness of the skin, nausea, muscle cramps, rash,
fatigue, diarrhea, and skin rashes.

Doctors haven't determined a safe point at which people with chronic
myelogenous leukemia can stop taking targeted drugs. For this reason,
most people continue to take targeted drugs even when blood tests reveal
a remission of chronic myelogenous leukemia.

Blood stem cell transplant
A blood stem cell transplant, also called a bone marrow transplant,
offers the only chance for a definitive cure for chronic myelogenous
leukemia. However, it's usually reserved for people who haven't been
helped by other treatments because blood stem cell transplants have
risks and carry a high rate of serious complications.

During a blood stem cell transplant, high doses of chemotherapy drugs
are used to kill the blood-forming cells in your bone marrow. Then blood
stem cells from a donor or your own cells that were previously
collected and stored are infused into your bloodstream. The new cells
form new, healthy blood cells to replace the diseased cells.

Chemotherapy
Chemotherapy drugs are typically combined with other treatments for
chronic myelogenous leukemia. Often, chemotherapy treatment for chronic
myelogenous leukemia is given as a tablet you take by mouth. Side
effects of chemotherapy drugs depend on what drugs you take.

Biological therapy
Biological therapies harness your body's immune system to help fight
cancer. The biological drug interferon is a synthetic version of an
immune system cell. Interferon may help reduce the growth of leukemia
cells. Interferon may be an option if other treatments don't work or if
you can't take other drugs, such as during pregnancy. Side effects of
interferon include fatigue, fever, flu-like symptoms and weight loss.

Clinical trials
Clinical trials study the latest treatment for diseases or new ways of
using existing treatments. Enrolling in a clinical trial for chronic
myelogenous leukemia may give you the chance to try the latest
treatment, but it can't guarantee a cure. Talk to your doctor about what
clinical trials are available to you. Together you can discuss the
benefits and risks of a clinical trial.

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Lifestyle and home remedies

For many people, chronic myelogenous leukemia is a chronic disease they
will live with for years. Many will continue treatment with imatinib
indefinitely. Some days, you may feel sick even if you don't look sick.
And some days, you may just be sick of having cancer. Self-care measures
to help you adjust and cope with a chronic illness include:

* Go to all of your medical appointments. People with chronic
myelogenous leukemia often face frequent medical appointments, blood
work and bone marrow exams. Appointments can bring anxiety because you
may fear your cancer is no longer in remission or your condition has
worsened. Expect some anxiety around the time of your appointments, but
don't let that stop you from going to each one. Plan stress-relieving
activities to take your mind off your anxieties. Try yoga, exercise and
time with friends and family.
* Talk to your doctor about your side effects. Powerful cancer
medications can cause many side effects, but those side effects often
can be managed with other medications or treatments. You don't
necessarily have to tough them out.
* Don't stop treatment on your own. If you develop unpleasant side
effects, such as skin rashes or fatigue, don't simply quit your
medication without consulting your health care professionals. Likewise,
don't stop taking your medications if you feel better and think your
disease may be gone. If you stop taking medication, your disease can
quickly and unexpectedly return, even if you've been in remission.
* Ask for help if you're having trouble coping. The emotional toll
of a chronic condition can feel overwhelming. Tell your doctor about
your feelings. You may get a referral to a counselor or other specialist
with whom you can talk.

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Alternative medicine

No alternative medicines have been found to treat chronic myelogenous
leukemia. But alternative medicine may help you cope with the stress of a
chronic condition and the side effects of cancer treatment. Talk to
your doctor about your options, such as:

* Acupuncture
* Aromatherapy
* Massage
* Meditation
* Relaxation techniques

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Coping and support

Chronic myelogenous leukemia often is a chronic disease and requires
long-term treatments. To help you cope with your cancer journey, try to:

* Learn enough about chronic myelogenous leukemia to make decisions
about your care. The term "leukemia" can be confusing, because it refers
to a group of cancers that affect the bone marrow and blood. Don't
waste time gathering information that doesn't apply to your kind of
leukemia. Ask your health care professionals to write down information
about your specific disease. Then narrow your search and seek out only
trusted, reputable sources, such as the Leukemia & Lymphoma Society.
* Seek out a specialist. Ask your doctor about his or her experience
in treating chronic myelogenous leukemia. If your doctor has little or
no experience in this area, ask about having a consultation with a
specialist. Most doctors aren't offended by this and may be willing to
help arrange a consult.
* Turn to family and friends for support. Stay connected to family
and friends for support. It can be tough to talk about your diagnosis,
and you'll likely get a range of reactions when you share the news. But
talking about your diagnosis and passing along information about your
cancer can help. So can the outpouring of practical help that often
results.
* Connect with other cancer survivors. Consider joining a support
group, either in your community or on the Internet. A support group of
people with the same diagnosis can be a source of useful information,
practical tips and encouragement.

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