Getting back to normal

Once it was a fatal cancer, but with the right medication CML is manageable, writes Aznim Ruhana Md Yusup

CANCER advocates celebrate World CML Day on Sept 22 to raise awareness on this rare disease called CML. It stands for Chronic Myeloid Leukaemia, and like most forms of leukaemia, it involves white blood cells. And “Chronic” in the name indicates that it is a slow, progressing cancer.

“In the lifetime of certain individuals, stem cells that make blood cells (in the bone marrow) attain a genetic mutation or abnormality known as the Philadelphia chromosome,” says Datuk Dr Chang Kian Meng, national head of haematology services and senior consultant haematologist in the haematology department of Ampang Hospital.

“It results in a molecular abnormality called Bcr-Abl, which gives rise to an abnormal protein known as tyrosine kinase that causes stem cells to make too many white blood cells. These cells then come into the blood circulation, so the person will have a very high white blood cell count.”

It’s well known that white blood cells help fight infection, but these cells don’t function so well. The raised white blood cell production also takes too much room in the bone marrow, so other blood cells — red blood cells and platelet — can’t be produced at a normal rate. Our body has 23 pairs of chromosomes. The Philadelphia chromosome takes

form on chromosome 22 when a section of chromosome 9 breaks off and sticks to it. This specific translocation between the chromosomes is the reason why World CML Day is celebrated on Sept 22.

SYMPTOMS AND PHASES

According to Dr Chang, CML patients in the west tend to be identified through routine blood tests on their annual health checkups. But since early detection awareness in the Asia Pacific is still quite low, most patients here are picked up through their symptoms.

“Often the patient will come because they feel a swelling or discomfort in the abdomen, because the liver and spleen are enlarged,” says Dr Chang. “Or they find that they lose their appetite because they have massive organs in their abdomen. Or they’re very tired because they’re anaemic, as a result of which then they get a full blood count.”

The danger of being diagnosed later is that there is a tendency for the condition to be at a more advanced stage, he adds. There are three stages to the disease; the early chronic phase is when patients have a raised white blood cell count but are still physically well.

“Most people remain in this stage for three to five years, after which the disease becomes inherently unstable. In the second stage called the accelerated stage, the white blood cell count gets even higher and they experience symptoms like fever and bone pain.”

“Finally, they enter a third stage known as the blast phase. The disease is now at the acute stage and this is the time that most patients will lose their lives because of bone marrow failure,” says Dr Chang.

TREATMENT AND GETTING WELL When a patient is referred to Dr Chang with CML symptoms, they are put through several tests to confirm the diagnosis. High white blood cell count alone isn’t enough because it can be caused by other things like injury or infection.

“So we need to do a chromosomal study to check if the patient has the Philadelphia chromosome. If you have this genetic abnormality, then you have CML.”

Once the diagnosis is confirmed, the patient will be counselled on their treatment options. The patient needs to continue to take their prescription even though they do not feel unwell anymore. They are also required to go for regular blood count checks to monitor the disease.