Have you ever felt very tired all of a sudden, had a few bruises, or had frequent bleeding gums? Although we don't pay much attention to these things, in rare cases, they could be signs of a more serious condition, such as Acute Promyelocytic Leukemia (APL) . So, don't worry, today we'll talk about this blood cancer called APL in a clear and simple way.
What is acute promyelocytic leukemia (APL)?
Simply put, APL is a very rare type of blood cancer . It actually belongs to a large group of leukemias called Acute Myeloid Leukemia . The main place where blood is made in our body is the bone marrow . Here in this bone marrow, due to a genetic change, that is, a genetic mutation , abnormal white blood cells start to form. These abnormal cells divide and multiply uncontrollably, spreading throughout the bone marrow. Sometimes doctors call this APL leukemia, or M3-leukemia .
APL is a serious condition. Its symptoms can come on suddenly and get worse quickly. Excessive bleeding is a major risk factor. But there is good news! With new treatments, including chemotherapy and new non-chemotherapy drugs, doctors are able to control APL and often cure it completely.
How common is this disease?
APL is actually a very rare disease . For example, in a country like the United States, only about 30,000 people are diagnosed with this disease each year. Most often, APL is diagnosed in people in their 30s .
What are the symptoms of APL?
APL symptoms occur when your bone marrow is unable to make healthy red blood cells , white blood cells, and platelets as normal. This reduction in all of these types of blood cells is called pancytopenia . When this happens, you can develop serious symptoms such as anemia , bleeding problems due to blood clotting , and frequent illnesses.
Other common APL symptoms are:
- Feeling extremely tired and exhausted due to a decrease in red blood cells (i.e. anemia).
- Frequent infections due to low white blood cells that fight disease. Things like fever and colds can occur frequently.
- Unintentional weight loss occurs because your body's metabolism speeds up and the energy from food is burned quickly.
Bleeding symptoms
A person with APL has a low number of platelets and blood clotting factors that help the blood clot. You know, platelets are what help stop or reduce bleeding. So, when these are low, the problem starts.
Here are some symptoms of bleeding caused by APL:
- Bleeding can occur anywhere in the body. For example, bleeding gums , frequent nosebleeds , and, in women, heavy menstrual bleeding .
- Blood collects under the skin and causes bruising in various places on the body . Even a small cut can cause a large bruise.
- Sometimes bleeding can occur inside the brain (intracranial hemorrhage) . If this happens, you may have difficulty moving your limbs, experience severe headaches, and may experience changes in your vision. This is an emergency!
- If your stool is black or has streaks of blood, it could be due to bleeding in the digestive system (gastrointestinal bleeding) .
What causes APL?
This disease is caused by two genes that control the production of our blood cells coming together to form an abnormal gene called PML-RARa . Importantly, this genetic mutation is not something you inherit from your parents . It happens randomly, meaning for no reason, during your lifetime. Experts still don't know exactly what causes this genetic change.
This mutation causes white blood cells to grow out of control, instead of developing properly, and instead form immature white blood cells (promyelocytes) . These immature cells fill the bone marrow, crowding out the space for healthy blood cells and platelets.
What are the complications of APL?
APL is a life-threatening condition. This is because the excessive bleeding that occurs can quickly become dangerous. If you cannot stop bleeding from even a minor cut, if you have a lot of blood in your stool or urine when you go to the toilet, or if you cannot stop bleeding from your gums, you should see a doctor or go to a hospital emergency room immediately .
Remember: If you have uncontrollable bleeding, never ignore it. Prompt treatment is key.
How is APL diagnosed?
Doctors usually perform several tests to diagnose this disease.
- Complete Blood Count (CBC): APL causes abnormal white blood cells to form. A CBC test can look at the different types of blood cells and the number of platelets in a sample of your blood.
- Peripheral Blood Smear: In this test, a blood sample is taken and examined under a microscope. The immature white blood cells (promyelocytes) are then checked for the presence of many small granules or special features called Auer rods . These are characteristic of APL.
- Bone Marrow Biopsy: In this test, a small sample of your bone marrow is taken and its cells are examined.
- Flow Cytometry: In this test, pathologists look for patterns of specific proteins on the surface of abnormal cells. These patterns can confirm APL.
- Polymerase Chain Reaction (PCR) test: This test can accurately detect the presence of the abnormal gene (PML-RARa) that causes APL.
- Cytogenetics: This tests for specific changes in the chromosomes of abnormal cells. Finding these changes is the main way to confirm the diagnosis of APL.
Doctors classify APL as low-risk or high-risk based on your white blood cell count. People with high-risk APL are more likely to have recurrent cancer or relapse .
How is APL treated?
APL is treated with a combination of differentiation agents , chemotherapy, and targeted therapy . In fact, since the discovery of these treatments in the 1980s, this disease, which was previously thought to be fatal, has now become a curable disease. This is a great achievement!
Cell differentiation drugs are non-chemotherapy drugs that help abnormal, immature white blood cells differentiate into normal, mature white blood cells. These non-chemotherapy drugs, listed below, have resulted in more than 95% remission and cure rates.
- All-trans-retinoic acid (ATRA) , or tretinoin (Vesanoid ®) – This is a form of vitamin A.
- Arsenic trioxide (ATO) – This is a form of arsenic.
If your doctor suspects you have APL, they may start you on ATRA even before other tests confirm the disease. This is because starting treatment early can reduce the risk of life-threatening bleeding.
Stages of APL treatment
Treatment is usually done in three phases: Induction, Consolidation, and Maintenance. Treatment may vary depending on the level of risk.
- Induction: The main goal of this phase is to kill enough leukemia cells to put your APL into remission . Remission means you have no symptoms and no signs of leukemia on tests. This is done by adding non-chemotherapy drugs, chemotherapy, and targeted therapies. During this time, you will need to stay in the hospital, usually for four to six weeks.
- Consolidation phase: Your oncologist may also call this ‘post-remission therapy’. This treatment keeps the APL in remission and continues to kill any remaining leukemia cells. This is the same medication that was used in the initial phase. This treatment can last for about eight months, with treatments every two months. You may have four weeks of treatment followed by four weeks off. This medication can be given as a pill or as an intravenous (IV) treatment .
- Maintenance phase: This is ongoing treatment, but the medication is given at a lower dose than in the initial and stabilization phases. This maintenance treatment is usually given for about a year.
Your oncologist may also administer supportive therapy , such as blood transfusions, along with this treatment.
Complications of treatment
The most common, and somewhat serious, complication is called differentiation syndrome . This is a group of severe reactions to APL medications. These reactions usually occur within the first three weeks of starting treatment. Symptoms can be mild or severe. Some of them include:
- Cough.
- Extra fluid around your heart and lungs (pleural effusion) .
- Renal failure .
- Low blood pressure (hypotension) .
- Decreased oxygen levels in the blood (hypoxemia) .
- Difficulty breathing (dyspnea) .
- Swelling/ inflammation of your arms, legs, and neck.
- Fever that comes without a reason.
- Weight gain for no reason.
If you develop this differentiation syndrome, your doctor may stop your treatment for a while. They may also use other medications, such as hydroxyurea, to lower your white blood cell count.
What can someone with APL expect?
In general, the prognosis is good . Although everyone's situation is different, studies have shown that between 90% and 95% of people with APL go into remission. However, APL can recur after treatment. Between 5% and 10% of people will have a relapse, usually within the first three years after treatment. They will then need further or different treatment.
Survival rates
Because APL is a rare disease, what we know about survival rates comes from clinical trials involving low-risk and high-risk APL patients.
One analysis shows that 99% of low-risk APL patients are alive four years after treatment. Another analysis of high-risk APL patients shows that 86% are alive five years after treatment. These are really good results!
How do I take care of myself?
Because APL can recur, it is very important that you see your doctor on time (follow-up appointments) .
You will have a medical check-up every month or two for the first year after treatment. After the first year, you will need to see your doctor about every three to four months for the next two years. Tests such as a CBC, PCR, and bone marrow biopsy may be done at these visits.
When should I go to the emergency room?
APL can recur after treatment, and symptoms can quickly get worse. If you are in remission from APL, go to the emergency room immediately if you experience any of the following:
- If you are bleeding uncontrollably .
- If you suddenly develop swelling with pain in your legs or lower abdomen .
Finally, an important message
Acute promyelocytic leukemia (APL) is a rare blood cancer that was once considered a fatal disease, but has now become a curable disease thanks to advanced treatments.
However, this is still a serious disease. If not diagnosed and treated promptly, it can be life-threatening . Therefore, if you have symptoms like uncontrollable bleeding, never ignore it. The most important thing is to seek medical advice and get a diagnosis as soon as possible. Don't be afraid, be courageous, because now there are good treatments for this!
` Leukemia, APL, Blood Cancer, Blood Cancer, Bone Marrow, Anemia, ATRA, Chemotherapy, Acute Promyelocytic Leukemia, M3-Leukemia, PML-RARa, Bleeding
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