Chronic myeloid leukaemia

ক্রনিক মায়েলয়েড লিউকেমিয়া (CML) কেন হয়?

CML ঠিক কী কারণে হয়, চিকিৎসকেরা তা নিশ্চিতভাবে জানেন না। তবে কিছু ঝুঁকির কারণ আছে, যা এই রোগ হওয়ার সম্ভাবনা বাড়িয়ে দিতে পারে।

বয়স

CML যেকোনো বয়সেই হতে পারে, তবে বয়স বাড়ার সঙ্গে সঙ্গে এটি বেশি দেখা যায়। যুক্তরাজ্যে CML ধরা পড়া রোগীদের অর্ধেকের বেশির বয়স 60-এর ওপরে।

লিঙ্গ

নারী ও জন্মের সময় মেয়ে হিসেবে চিহ্নিত ব্যক্তিদের তুলনায় পুরুষ ও জন্মের সময় ছেলে হিসেবে চিহ্নিত ব্যক্তিদের মধ্যে CML সামান্য বেশি দেখা যায়, যদিও এর কারণ জানা যায়নি।

তেজস্ক্রিয়তার সংস্পর্শ

খুব বেশি মাত্রার তেজস্ক্রিয়তার সংস্পর্শে এলে, যেমন কোনো পারমাণবিক দুর্ঘটনার পর, CML হওয়ার ঝুঁকি বেড়ে যায়। যুক্তরাজ্যে খুব কম মানুষই এত বেশি মাত্রার তেজস্ক্রিয়তার সংস্পর্শে আসেন, যাতে CML-এর ঝুঁকি বাড়তে পারে।

গবেষণায় CML হওয়ার ঝুঁকির সঙ্গে নিচের বিষয়গুলোর কোনো সম্পর্ক পাওয়া যায়নি:

• পারমাণবিক বিদ্যুৎকেন্দ্রের কাছে বসবাস করা
• ইলেকট্রোম্যাগনেটিক ফিল্ডের (তড়িৎচুম্বকীয় ক্ষেত্র) সংস্পর্শে আসা
• উচ্চ ভোল্টেজের বিদ্যুতের তারের কাছে বসবাস করা
• ঘরের ভেতরের রেডন, যা একটি প্রাকৃতিকভাবে সৃষ্ট গ্যাস

Diagnosing chronic myeloid leukaemia (CML) and planning treatment

If you have symptoms, you will usually begin by seeing your GP, who will examine you. If they think you may have chronic myeloid leukaemia (CML), they will refer you to a haematologist for further tests. A haematologist is a doctor who specialises in diagnosing and treating blood problems.

The haematologist will ask you about any illnesses or health problems you have had. They will check if your spleen is enlarged and arrange tests.

You may have the following tests.

Blood tests

You will have blood tests at the hospital. These tests will:

• check the numbers of blood cells in your blood (this is called a full blood count, or FBC)
• look for leukaemia cells

If there are leukaemia cells in your blood, the haematologist will arrange more tests. These will find what type of leukaemia you have and what phase it is. This will help your doctors plan your treatment.

Bone marrow biopsy

A bone marrow biopsy is when a doctor or nurse takes a small sample of bone marrow to test for abnormal white blood cells. They usually take the bone marrow from the back of the hip bone (pelvis). Rarely, they may take it from the breast bone (sternum).

Ultrasound scan

You might have an ultrasound scan to check the size of your spleen and liver. Ultrasound scans use sound waves to build up a picture of the inside of the body. This is a painless test that takes around 10 to 15 minutes.

Cytogenetic and molecular tests

Cytogenetic and molecular tests look for gene changes (mutations) inside the leukaemia cells. Tests may include:

Philadelphia chromosome test

Doctors use the samples of blood and bone marrow they have taken to look for the Philadelphia chromosome.

Polymerase chain reaction (PCR) test

This blood test looks for the BCR-ABL1 gene in leukaemia cells. It is a very sensitive test that can detect tiny amounts of leukaemia.

If you are diagnosed with CML, you will have regular PCR blood tests. Doctors use this test to check how well treatment is working.

PCR tests can also help identify additional gene changes, called mutations, in the BCR-ABL1 gene. Some of these gene changes can tell your doctor how targeted therapy drugs are likely to work for you. These drugs are called tyrosine kinase inhibitors (TKIs). A change they will look for is the T3151 gene change.

PCR tests can detect very small amounts of BCR-ABL, even if cytogenetic testing does not find the Philadelphia chromosome.

FISH testing

Fluorescence in situ hybridisation (FISH) can be used to find:

• specific chromosome abnormalities
• other genetic changes in CML cells

This information can help your doctor plan your treatment.

Waiting for test results can be a difficult time. Macmillan Cancer Support have more information that can help.

Phases of chronic myeloid leukaemia (CML)

The treatment you have depends on the phase of chronic myeloid leukaemia (CML). CML usually develops slowly.

The 3 phases of CML are the:

• chronic phase
• accelerated phase
• blast phase

Doctors know the phase of CML from:

• the number of immature blood cells (blast cells) in your blood and bone marrow
• your symptoms

Most people are diagnosed when CML is in the chronic phase.

Chronic phase

When CML is in the chronic phase, there may be no symptoms. Most people can live a normal life. Treatment is tablets you can take at home. You will have regular blood tests to check how well the treatment is working.

For most people, the leukaemia can be well controlled for as long as treatment continues.

In the chronic phase, less than 15 in 100 blood cells in the blood or bone marrow (15%) are blast cells.

Accelerated phase

In a small number of people, the leukaemia may go from the chronic phase to the accelerated phase. This can happen if the CML does not respond well to treatment. Sometimes people are diagnosed with CML in the accelerated phase.

In the accelerated phase, less than 20 in 100 blood cells in the blood or bone marrow (10% to 19%) are blast cells.

In this phase, there are more blast cells in the blood or bone marrow. You may also develop some of the more common symptoms of leukaemia.

If you feel unwell or develop new symptoms, tell your doctor.

Blast phase

For some people, CML in the accelerated phase may go into the blast phase. Rarely, people are diagnosed with CML in the blast phase. Or the leukaemia may change straight from the chronic phase to the blast phase.

The blast phase is like an acute leukaemia. In this phase, more than 20 in 100 blood cells in the blood or bone marrow (20%) are blast cells. The blast phase is sometimes called blast crisis.

You will likely develop symptoms because the bone marrow is not making enough healthy blood cells.

Macmillan Cancer Support have more information about symptoms of CML.

Relapse

Relapse means the leukaemia cells have come back after a time in remission (where there are no signs of leukaemia cells). In CML, this is usually found using the PCR test.

Risk score of chronic myeloid leukaemia (CML)

As well as the phase of CML, your cancer doctor will calculate your risk score. This can help them to plan the best treatment for you.

Risk scores are based on:

• your age
• the size of your spleen
• the number of blast cells in your blood
• your platelet count

Depending on the risk score, people with CML can be grouped into 3 risk groups:

• low
• intermediate
• high

Knowing the risk group can give your haematologist an idea of how likely the CML is to respond well to treatment.

Most people with CML respond to tyrosine kinase inhibitor drugs (TKIs) regardless of their risk score.

Doctors bring together a team of specialists to work out the best possible treatment plan for you. This is called a multidisciplinary team, or MDT.

Your haematologist or specialist nurse will walk you through the different treatment options and their side effects. They will also talk with you about the things you need to weigh up when deciding on treatment. You should have all this information in hand before you give your consent to start any treatment.

The goal of treatment is to bring CML into remission and keep it there. This means your PCR blood test shows no signs of CML, and you feel well. Remission itself has different levels.

Treatment options may include the following.

Tyrosine Kinase Inhibitors (TKIs)

The main treatment for CML is a group of drugs called tyrosine kinase inhibitors, or TKIs.

Chemotherapy

Chemotherapy uses anti-cancer (cytotoxic) drugs to destroy or damage leukaemia cells. It works by interfering with the way leukaemia cells grow and divide.

Stem cell transplant

Your doctors may raise the possibility of a stem cell transplant, sometimes called a bone marrow transplant. This is an intensive treatment that is not suitable for everyone, and it is not often used to treat CML.

Interferon alpha

If other treatments have not worked, some people may be given interferon alpha during the chronic phase of CML.

Doctors may also turn to interferon alpha for people who need CML treatment and are planning a pregnancy, or who are already pregnant.

Leukapheresis

At the time you are diagnosed with CML, you may have a very high number of white blood cells in your blood. Doctors can bring this down using a process called leukapheresis.

You may receive some treatments as part of a clinical trial.

Macmillan Cancer Support have more information about treatment for CML.

Fertility and chronic myeloid leukaemia (CML) treatment

Some CML treatments can affect your fertility or harm a developing baby. Because of this, you may be advised to use contraception to avoid becoming pregnant during treatment.

If you are worried about your fertility, it is important to talk with your doctor before treatment begins.

Macmillan Cancer Support have more information on cancer treatment options and on getting organised before cancer treatment.

After treatment with a tyrosine kinase inhibitor (TKI), your healthcare team will keep a close eye on you, usually seeing you every 1 to 2 weeks at first.

Your doctor will also arrange a polymerase chain reaction (PCR) blood test every 3 months in the beginning, to check for any remaining leukaemia cells. If you are responding well to TKI treatment, this test may be needed less often over time.

From time to time, your doctor may also want to take a small sample of your bone marrow. They will let you know how often this is likely to be necessary in your case.

As you continue to do well, your check ups may eventually be spaced out to every 3 to 6 months.

If you are having a different type of treatment, your doctor or nurse will explain how often you should expect to come in for appointments. Between visits, if you notice any new symptoms or have concerns, do not wait for your next appointment. let your doctor or specialist nurse know as soon as possible.

Wellbeing and recovery

Most people living with chronic myeloid leukaemia (CML) can expect a normal life expectancy. To support your health going forward, it can help to look closely at your day to day habits and see where small improvements are possible. This is worth doing even if you already followed a fairly healthy lifestyle before your diagnosis.

Simple, steady lifestyle changes can support your recovery and are often easier to keep up than big ones. Even modest adjustments to diet, activity or rest can make a real difference to how you feel and to your long term health.

If you would like more detailed guidance on building healthy habits after treatment, Macmillan Cancer Support has further information on this.

Getting support

There is no single right way to cope with a diagnosis like this, and people manage the emotions that come with it differently. Many find it helpful to talk things through, whether with family, friends, or your doctor or nurse.

Macmillan Cancer Support can also offer further help. You can:

• call the Macmillan Support Line on 0808 808 00 00
• chat with a Macmillan specialist online
• join the chronic myeloid leukaemia (CML) forum, where you can connect with others affected by CML, ask questions and share experiences

A few other organisations also offer information and support:

• CML Support, which offers information and support to patients and to the families and carers of those affected by CML
• Anthony Nolan, the UK's largest stem cell and bone marrow register
• Leukaemia CARE, which offers care and support to those affected by leukaemia, lymphoma or a related blood disorder, along with their families and carers
• Blood Cancer UK, a blood cancer research charity offering information and support for any type of blood cancer

When reaching out for support, it is best to avoid sharing personal details such as your name, location or other health conditions.