Chronic Myelogenous Leukemia

Chronic myelogenous leukemia (also called chronic myeloid leukemia or CML) is a type of cancer that affects blood cells and bone marrow, the spongy tissue inside bones where blood cells are produced.

The name "myelogenous" comes from myelocyte, a kind of immature white blood cell. White blood cells are the immune system cells that help your body fight infection. CML has three phases: chronic, accelerated, and blastic.

When you have chronic phase CML, you're in the earliest phase of the disease. You might not even realize that you have CML until you're diagnosed. Many people in this phase of CML do not have any symptoms.

In the accelerated phase, the number of abnormal blood cells in your body increases. People in this phase usually have symptoms such as: 

• Fatigue
• Fever
• Bruising
• Night sweats
• Shortness of breath
• Unexplained weight loss
• Appetite loss
• Swelling or pain on your left side (which could be a sign of an enlarged spleen)
• Bone pain

Other side effects may include stroke, visual changes, ringing in the ears, stupor, and prolonged erections (priapism).

When CML reaches the blastic phase, the abnormal cells have multiplied to the point where they're crowding out healthy white blood cells, red blood cells, and platelets. People who are in the blastic phase experience more severe symptoms, including infections, bleeding, skin lesions, swollen glands, and bone pain. This is the most serious phase of CML.

Your doctor will use medication or other treatments to keep your CML in the chronic phase, when it's easiest to treat.

What Causes CML?

CML starts in your genes. Each human cell contains structures called chromosomes, which carry the genes (DNA). Genes are the codes that instruct cells to produce certain proteins.

For some reason (which researchers haven't yet pinpointed), in CML, a section of chromosome 9 switches places with a section of chromosome 22. The combination produces a new chromosome, called the Philadelphia chromosome (named after the city where it was discovered). 

The Philadelphia chromosome creates a new gene, called BCR-ABL. This gene instructs blood cells to produce a protein called tyrosine kinase.

Tyrosine kinase causes your body to make too many white blood cells. These white blood cells are immature. They don't work as well as normal white blood cells. As a result, your body can't fight infection as well as it used to. The extra abnormal white blood cells leave less room for healthy white blood cells, red blood cells, and platelets.

Learning That You Have CML

You might have no idea you have CML until your doctor discovers it during a routine blood test. About half of people who are diagnosed with chronic phase CML don't have any symptoms. 

If your doctor suspects that you have chronic phase CML, tests can be done to confirm the diagnosis. These tests include: 

• A complete blood count (CBC) to determine how many white blood cells, red blood cells, and platelets you have
• Bone marrow aspiration and biopsy, in which a sample of bone marrow and blood are examined to determine how advanced the cancer is
• Fluorescence in situ hybridization (FISH) to measure the number of cells containing the Philadelphia chromosome
• CT scans to evaluate the size of your spleen
• Polymerase chain reaction (PCR) looks for the BCR-ABL cancer gene in your cells.

What Happens Next?

Now that you’ve been diagnosed with CML, you may not know what to do next. To make sure you're getting the most expert care, you should see a hematologic oncologist, a doctor who specializes in treating CML. If you don’t already have a specialist, ask your primary care doctor for a referral or check with a cancer organization such as the Leukemia & Lymphoma society.

The first step is to learn as much as possible about your condition. CML will seem a lot less scary and a lot more manageable once you understand it better. Ask your treatment team for brochures or other printed materials. Or look for information on reputable web sites like the American Cancer Society or the Leukemia & Lymphoma Society.

Don't be afraid to ask questions. You need to understand your condition and how it will be treated, so that you can make important decisions about your own care and treatment.

Write down a list of questions to bring with you to your doctor’s appointments. Ask questions about your condition and treatment plan, such as:

• What are my treatment options?
• Which treatment is best for me?
• What kinds of results can I expect from CML treatment?
• What side effects might I experience from CML treatment?
• Do I need a second opinion?

Your goal is to stay in the chronic CML phase for as long as possible and remain symptom-free. You can help your doctors achieve that goal by:

• Keeping all of your medical appointments
• Following the treatment plan your doctor prescribed
• Keeping your doctor updated on any treatment-related side effects you’re experiencing
• Telling your doctor if any new symptoms develop
• Asking questions about your treatment plan if you are unclear about any aspects

CML Treatment

The goal of CML treatment is to destroy the abnormal blood cells in your body, and return the number of healthy blood cells to normal.

Typically, CML treatment starts with drugs known as tyrosine kinase inhibitors (TKI), which work to block the action of tyrosine kinase. 

• The most common and oldest drug for initial therapy is imatinib (Gleevec). 
• The drugs dasatinib (Sprycel) and nilotinib (Tasigna) are FDA-approved for the initial treatment of CML. 
• Bosutinib (Bosulif) and ponatinib (Iclusig)  are other TKIs and can be used if a person is resistant to or cannot tolerate the side effects of other drugs. 
• Yet another drug, Synribo (omacetaxine mepesuccinate), is approved for those whose disease has progressed after using two or more TKIs.

Other CML treatment options include a stem cell transplant, chemotherapy, such as hydroxyurea or busulfan, or biologic therapy with interferon.

Stem cell transplantation is an option when other therapies have failed. Stem cell transplantation for CML requires a matching donor.

Chronic phase CML is very treatable, and once you have been treated, you should be able to go on with your normal life. It's important to treat CML at this phase, so that it doesn't progress to the more serious accelerated or blastic phases.

Your doctor will tell you which treatment is best for you based on your CML phase. Be an informed patient and talk to your doctor if you have any concerns or questions during the course of treatment.

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