What is Chronic Lymphocytic Leukemia?
Lymphocytic or Chronic Lymphatic Leukemia (CLL) also known as chronic lymphoid leukemia is a blood cancer that develops when the body produces too many white blood cells in an abnormal way. This occurs because they live too long or multiply extremely quickly, causing large amounts to circulate in the blood.
During this process they exclude normal white blood cells and do not fight infection themselves, so there may be an increased risk of infection (neutropenia). As the leukemia progresses, the bone marrow fills with leukemia cells and there is less room for healthy red blood cells and platelets to be made.
This can cause various health problems, such as anemia (from too few red blood cells) or bleeding or bruising (from thrombocytopenia, too few platelets).
What is the difference between chronic and acute leukemia?
All types of leukemia start in the bone marrow and affect the production of white blood cells. They are classified according to the type of white blood cell affected (lymphoid or myeloid), abnormalities in the bone marrow, and how quickly the disease develops.
Chronic leukemia usually affects partially immature cells, appears gradually, and develops slowly over months or years.
Acute leukemia affects completely immature cells, occurs suddenly and develops rapidly.
Who gets LLC?
Each year in Australia, approximately 3,700 people are diagnosed with a form of leukemia and more than 1,700 of these cases are chronic leukemia.
Chronic lymphocytic leukemia is the most common type of chronic leukemia, with approximately 1,400 people diagnosed each year. It is twice as likely in men as in women and is very rare in children.
What Causes Chronic Lymphatic Leukemia?
Chronic lymphocytic leukemia is caused by changes in one or more of the genes (DNA) that control the growth and development of blood cells. These changes occur over time, but it is not known why they occur in some people and not in others.
The exact cause of chronic lymphatic leukemia is still unknown. Some people have genetic abnormalities that can lead to this disease, these genetic defects are generally not inherited, but there are rare cases in which CLL can run more repeatedly in families.
If you are concerned about this, talk to your doctor, who can refer you to a genetic counselor.
Blood cells and leukemia
Blood is pumped around your body to provide your tissues with oxygen and nutrients, as well as to remove waste products. It is made up of blood cells that are carried by a clear fluid called plasma.
The three main types of blood cells have specific functions:
- Red blood cells: carry oxygen throughout the body.
- White blood cells: fight infection.
- Platelets: help blood clot.
These blood cells have a limited lifespan and must be continually replaced.
Most are made in the bone marrow, which is the spongy part in the center of the bones and contains stem cells, which are unspecialized blood cells.
These stem cells first develop into immature cells known as blast cells. Normally, blast cells develop into mature red or white blood cells. They can also transform into platelets and carry out their established functions.
There are two families of stem cells:
- Myeloid Stem Cells: develop into myeloblast cells and then into red blood cells. They are most types of white blood cells and platelets.
- Lymphoid Stem Cells: They become lymphoblastic cells and then lymphocytes, which are a type of white blood cell.
If myeloblast or lymphoblast cells do not mature properly or there are too many in the blood, they can cause leukemia.
In leukemia, the blast cells never develop into mature white blood cells. When this happens, they are called leukemia cells.
The lymphatic system
The lymphatic system works with white blood cells to protect the body from infection. A large network of thin tubes (lymphatic vessels) carries a clear fluid called lymph.
Lymph travels to and from areas of lymphatic tissue, including the lymph nodes, spleen, and liver. When leukemia causes abnormal white blood cells to accumulate, the lymphatic tissue becomes inflamed.
Lymph nodes: These are small, bean-shaped structures found in the neck, armpits, chest, abdomen, and groin. These filter toxins and help fight infection. They also make some blood cells.
Spleen – This is an organ on the left side of the body below the ribs. Eliminate old or damaged blood cells.
Liver: This large organ removes toxins, controls sugar levels, and stores vitamins.
Symptoms of CLL
Chronic lymphocytic leukemia usually doesn’t cause any symptoms early on and can only be taken during a blood test done for another reason.
When symptoms develop, they can include:
- Get infections often.
- Bleeding and bruising more easily than normal.
- A high temperature (fever).
- Night sweats.
- Swollen glands in the neck, armpits, or groin.
- Bloating and discomfort in your stomach.
- Involuntary weight loss
You should visit your doctor if you have persistent or worrisome symptoms. These symptoms may have causes other than cancer, but it is a good idea to get them checked out.
Treatment for chronic lymphocytic leukemia depends largely on what stage the condition is in when it is diagnosed. You may only need to be watched at first if you are caught early on.
On the other hand, if it is in a more advanced stage, then chemotherapy is the main treatment that can often help keep chronic lymphocytic leukemia under control for many years.
It may go away after treatment initially (called remission) but will usually reappear a few months or years later and may need to be treated again (this is known as a recurrence).
Stages of Chronic Lymphocytic Leukemia
Doctors refer to “stages” to describe how far chronic lymphocytic leukemia has developed and to help them determine when it needs to be treated.
There are three main stages:
- Stage A : You have enlarged lymph nodes in fewer than three areas (such as the neck, armpit, or groin) and a high white blood cell count.
- Stage B : You have enlarged lymph nodes in three or more areas and a high white blood cell count.
- Stage C : You have enlarged lymph glands or an enlarged spleen, a high white blood cell count, and a low red blood cell or platelet count.
Stages B and C are usually treated right away. Stage A usually only needs treatment if it gets worse quickly or begins to cause symptoms.
Early Stage LLC Monitoring
Treatment may not be necessary if you do not have any symptoms when you are diagnosed with chronic lymphocytic leukemia.
This is because:
- As mentioned before, CLL often develops very slowly and may not cause symptoms for many years.
- There is no benefit in starting treatment early.
- Treatment can cause significant side effects.
In these cases, you will usually only need regular visits to your doctor and blood tests to monitor the condition.
Chemotherapy treatment will usually only be recommended if you develop symptoms or tests show the condition is getting worse.
Chemotherapy for more advanced Chronic Lymphatic Leukemia
Many people with CLL will eventually need chemotherapy. This involves taking medications to keep the cancer under control.
There are several different medications for chronic lymphocytic leukemia, but most people will take three main medications in treatment cycles that last 28 days. These medications are:
- Fludarabine – Usually taken as a tablet for three to five days at the beginning of each treatment cycle.
- Cyclophosphamide : It is also usually taken as a tablet for three to five days at the beginning of each treatment cycle.
- Rituximab : It is given into a vein over a few hours (intravenous infusion) at the beginning of each treatment cycle.
Fludarabine and cyclophosphamide can usually be taken at home. Rituximab is given in the hospital, and sometimes you may need to stay in the hospital overnight.
Other different medications may also be tried if you cannot take the ones listed above, if you have tried them but they have not worked, or if your CLL has returned after treatment.
These include bendamustine, chlorambucil, ibrutinib, idelalisib, obinutuzumab, ofatumumab, and prednisolone.
Side effects of this treatment
Medications used to treat chronic lymphocytic leukemia can cause some serious side effects, including:
- Persistent tiredness
- Feeling sick.
- An increased risk of infections.
- Easy bruising or bleeding.
- Hair loss or thinning.
- An irregular heartbeat
- Allergic reactions.
Most side effects will pass after treatment ends. Let your care team know if you experience any side effects, as there are some treatments that can help.
Stem cell or bone marrow transplants
Stem cell or bone marrow transplants are sometimes used to try to completely eliminate chronic lymphocytic leukemia or to control it for longer periods of time.
A stem cell transplant involves:
- Receive high doses of chemotherapy and radiation therapy to kill cancer cells in your body.
- Remove stem cells from a donor’s blood or bone marrow; ideally, it will be someone close to you, such as a sibling.
- Transplant the donor stem cells directly into one of your veins.
This is the only possible cure for chronic lymphatic leukemia, but it is not applied very often as it is intensive treatment and many people with the disease are older and in poor condition for the benefits to outweigh the risks.
Initial treatment with chemotherapy and radiation therapy can put significant pressure on your body and cause troublesome side effects.
There is also a risk of serious problems after transplantation, such as graft-versus-host disease in which the transplanted cells attack the other cells in your body.
Other treatments for Chronic Lymphocytic Leukemia
There are also other treatments that are sometimes used to help treat some of the problems caused by CLL, particularly if you cannot have chemotherapy or if it does not work.
- Radiation therapy to shrink lymph glands or a swollen spleen.
- Surgery to remove a swollen spleen.
- Antibiotics, antifungals, and antiviral medications to help reduce the risk of getting an infection during treatment.
- Blood transfusions to provide more red blood cells and platelets (clotting cells) if you experience severe anemia or bleeding and bruising problems.
- Immunoglobulin replacement therapy – a transfusion of antibodies taken from donated blood that can help prevent infection.
- Injections of drugs called granulocyte colony stimulating factor (FSC-G) to help increase the number of white blood cells.
You may also need additional treatment for any complications of the disease that develop.
Decide against treatment
Since many of the treatments for chronic lymphatic leukemia can have unpleasant side effects that can affect your quality of life, you may choose not to have a particular type of treatment.
This is your decision and your treatment team will respect any decision you make. Don’t be too quick to decide on your treatment, and before making a decision you can talk to your doctor, partner, family and friends. Pain relief and nursing care will continue to be available when you need it.
Clinical trials for CLL
Several clinical trials are currently underway in the UK to try to find the best way to treat chronic lymphocytic leukemia. These are studies that use new and experimental techniques to see how well they work in treating and possibly curing the condition.
If you are interested in participating in a clinical trial, your care team can tell you if there is one taking place in your area and explain the benefits and risks involved.