Many of us are afraid of the word "cancer." It's a very common thing. But today, because medical science has advanced so much, it has become possible to live normally with some types of cancer. Today, we are going to talk about one such type of blood cancer. That is CML, which stands for Chronic Myeloid Leukemia. You may have heard this name after a routine blood test. So, let's talk about it in detail without being afraid.
Okay, so what is this CML?
Simply put, CML is a cancer of our blood. It starts in the bone marrow inside our bones. The bone marrow is like a factory that makes the types of cells our body needs called red blood cells, white blood cells, and platelets. In CML, something goes wrong with the myeloid stem cells in these bone marrow cells, and they start to divide and multiply uncontrollably.
This is also called `chronic myelogenous leukemia` or `chronic granulocytic leukemia`.
But the most important and best news here is that with today's advanced treatments, most people with CML can live a normal life expectancy . This means that instead of being a fatal disease, it has become a chronic condition that can be controlled with medication.
How quickly does this CML progress?
The word "chronic" means "long-term." And that's what this disease is like. CML is a very common disease. Sometimes it can be present for years without any symptoms. Many people find out they have it by accident, when a blood test done for another reason shows an abnormality in their blood cell count.
But if left untreated, this can become a life-threatening condition within three to four years. Therefore, it is very important to diagnose and start treatment early.
What symptoms might someone with CML experience?
Often, there may be no major symptoms in the early stages. However, there are some common symptoms that appear over time. These are often things that we think are normal and ignore.
| Symptom | A simple explanation |
|---|---|
| Constant fatigue and weakness | Always feeling tired, even if you sleep well and don't do any work. |
| Difficulty breathing (Dyspnea) | Falling while walking a short distance or climbing stairs. |
| Unexplained fever | Feeling hot without any other illness. |
| Night sweats | Sweating at night to the point where the sheets get wet, even though the room is cold. |
| Weight loss for no reason | Losing weight suddenly without dieting or exercising. |
| Swelling or discomfort in the upper left part of the abdomen | This is due to swelling of the spleen, which is located in this part of the abdomen. |
| Feeling full even after eating a little | A swollen spleen can press on the stomach, making you feel full even after eating a small amount. |
Why does CML develop? Is it hereditary?
This question is something that many people have. The most important thing to understand here is that CML is not a hereditary disease . This means that children do not develop it just because their parents had it. And it is not something that is inherited.
CML occurs when a change (mutation) occurs in the genes of our cells, namely the stem cells in the bone marrow, during our lifetime. This is called an `acquired mutation`.
Okay, now let's see how this works. Simply put, it's like this:
1. A change occurs in the genes of our stem cells, and a new, added gene called `BCR-ABL` is formed.
2. This new gene gives new instructions to stem cells to make an unusual form of an enzyme called `tyrosine kinase`.
3. Think of these enzymes as like the "on" and "off" switches on a light. These are what control cell division.
4. But that abnormally formed `tyrosine kinase` enzyme doesn't have an "off" switch. It's always "on".
5. Because there is no "off" switch, the stem cells in the bone marrow continue to receive signals to divide, divide, and multiply uncontrollably.
6. As a result, a large number of immature white blood cells (also called `blasts`) begin to form.
7. Over time, these abnormal cells fill the bone marrow. Then the bone marrow loses its ability to make healthy red blood cells, white blood cells, and platelets. As a result, the symptoms mentioned above begin to appear.
The risks and possible complications of this
Risk factors
The only known risk factor for developing CML is exposure to very high levels of radiation . However, this is very rare. The levels of radiation we encounter in normal life (for example, from getting an X-ray) do not cause it.
Possible complications
CML can cause two main complications:
- Anemia: A decrease in the production of healthy red blood cells, which reduces the amount of oxygen the body needs, causing fatigue and paleness.
- Splenomegaly: A large number of abnormal blood cells accumulate in the spleen, causing it to swell. This can cause stomach discomfort and a feeling of fullness even after eating a small amount.
In addition, some studies have shown that people with CML may have a slightly increased risk of developing other types of cancer (second cancers).
How do doctors diagnose CML?
Your doctor may suspect this with a simple blood test. However, to confirm the disease, special tests that look for changes in genes are needed.
| Test | What do you see in this? |
|---|---|
| Complete Blood Count (CBC) | They check to see if the white blood cell count is too high or if the red blood cell count is too low. |
| Bone Marrow Aspiration/Biopsy | A small sample of bone marrow is taken and tested to see if the genetic mutation called `BCR-ABL` is present. |
| CT Scan (Computed Tomography Scan) | Check to see if the cancer has spread to other parts of the body (e.g. spleen, liver). |
| Ultrasound Scan | This test is used to see if the spleen is larger than normal. |
What are the phases of CML?
Unlike other cancers, CML is not classified as "stages 1, 2, 3, 4." Instead, it is classified as "phases." This is determined by the number of immature white blood cells (`blasts`) in the blood and bone marrow.
- Chronic Phase: This is the earliest stage. There are less than 10% of `blasts` cells in the blood and bone marrow. 80-90% of people diagnosed with CML are in this stage. These people may or may not have symptoms.
- Accelerated Phase: In this phase, the number of `blasts` cells increases to between 10% and 19%. Symptoms begin to become more noticeable.
- Blast Phase or Blast Crisis: This is the most severe and life-threatening phase. The number of `blasts` cells increases by 20% or more. Symptoms such as extreme fatigue, fever, and weight loss become severe.
- Resistant CML: This is the name given to CML that does not respond to treatment or that recurs after treatment.
What are the treatments for CML?
This is where the good news about CML comes in. Today, there are very effective treatments for this disease.
The main treatment is a class of drugs called Tyrosine Kinase Inhibitors (TKIs) . These are medications that are taken as pills.
Remember the unusual enzyme that we talked about earlier that acts like an "on" switch? That's what these drugs target. What TKIs do is they stop that enzyme that doesn't have an "off" switch from working. They block it. Then those uncontrollably dividing CML cells don't get the signal to divide, and after a while those cells die.
These TKIs have made a huge difference in the lives of CML patients. Before these drugs came along, only about 20% of people diagnosed with CML survived for 5 years. But today, with TKIs, that percentage has increased to over 90%.
These medications are often taken for life. Some of the commonly used types of TKIs are:
- Imatinib
- Dasatinib
- Nilotinib
- Bosutinib
- Ponatinib
Do TKIs have side effects?
Yes, like any medicine, there can be side effects. But they can usually be managed. The most common ones are:
- Stomach ache
- Tiredness
- Diarrhea
- Muscle rolling
- Edema
- Sometimes, things like liver damage and low blood cell counts can occur.
Your doctor will talk to you about these side effects and help you manage them.
Is CML completely curable?
At this point, the only way to "cure" CML is with an allogeneic stem cell transplant . This involves transplanting stem cells from a healthy person into the patient. However, this is a very complex and risky treatment. Therefore, it is usually only done for patients who do not respond to TKIs.
What is it like to live with CML and what is the outlook?
When CML goes into remission with treatment, you no longer have symptoms and no signs of the disease can be detected on tests. This means you can live a normal life. But you will need to continue taking medication to control the disease. You will also need to see your doctor regularly for blood tests to check how well your treatment is working.
Treatment-Free Remission (TFR)
This is the latest and most promising development in CML treatment. This means that some patients who have been taking TKIs for a while and whose disease has been very well controlled can now stop taking the medication under the supervision of their doctor. Even after stopping the medication, the disease has not returned. But this is not possible for everyone. You have to meet certain qualifications for it.
The most important thing is, never stop taking your medication for any reason without talking to your doctor. That can be very dangerous.
Important questions to ask your doctor
When you find out you have CML, it's normal to have a lot of questions in your mind. Don't be afraid, ask your doctor about these things.
- What stage of CML do I have?
- What treatment options do I have?
- How will I feel during treatment? What are the side effects?
- Can I continue my job while receiving treatment?
- How often do I need to have blood tests to see if the treatment is successful?
- What are the chances of my disease going into remission?
- Is a stem cell transplant suitable for my condition?
- Who can I talk to about managing treatment costs?
Take-Home Message
- CML is a slow-growing blood cancer. It is not hereditary.
- Today's targeted therapies, called TKIs, are very effective, allowing many people with CML to live normal, long lives.
- Once you are diagnosed with the disease, don't panic and follow your doctor's instructions exactly.
- It is essential to take medication on time and go to clinics for checkups on time.
- Never stop or change treatment without your doctor's approval.
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