Chronic Lymphocytic Leukemia: Prognosis and Outlook

The average age of onset for chronic lymphocytic leukemia is 70. CLL starts in one of the types of white blood cells found in the bone marrow. White blood cells are an integral part of your immune system. They help you stave off infection and disease. CLL is uncommon in people under the age of 40. Men are slightly more likely to get this condition than women.

This article will discuss survival rates and what you can expect if you have this condition. It will also touch upon CLL treatments, coping mechanisms you can try, and factors that impact CLL prognosis.

In CLL, too many lymphocytes (a type of white blood cell) are produced from a single cell line (monoclonal). These blood cells crowd out healthy white blood cells, as well as red blood cells and platelets.

CLL is a slow-growing cancer that spreads from the bone marrow into the bloodstream. Eventually, it may spread into other areas of the body, including the liver, lymph nodes, and spleen.

People with CLL may not have any symptoms for years. Once they occur, symptoms may include:

• Swollen lymph nodes in the armpits, neck, groin, and stomach
• Night sweats
• Inability to stave off repeated infections
• Getting sick easily with conditions such as colds and the flu
• Exhaustion and fatigue
• Enlarged spleen that causes pain
• Unexplained weight loss
  

Chronic Lymphocytic Leukemia Survival Rate

The survival rate for CLL is better than for many other types of cancer. Data compiled by the National Cancer Institute (NCI) from 2011 to 2017 indicate that the five-year relative survival rate for CLL is 87.2%. NCI data for that same time frame show a decline in the new case rate and death rate for CLL.

It’s important to remember that relative survival rates are estimates based on data compiled from large numbers of people with a common diagnosis. This statistic does not take treatments or responses to treatments into account and is not predictive of what will happen in your situation.  

Factors That Influence Outlook

No two people are alike. Your CLL prognosis will vary from that of other people with this condition. Age, overall health, and response to treatment can all have an impact on your outlook and what you can expect. 

Diagnosis of CLL

CLL symptoms may not surface for years. They can also indicate many other conditions. Even so, it’s important to let your doctor know if you have any CLL symptoms. This can help streamline your diagnosis.

To diagnose CLL, your doctor will conduct a physical exam to look for lymph node swelling and abdominal pain. They’ll also take a medical history that includes information about your family’s history of this disease.

They’ll want to know about toxin exposure you may have had to herbicides, including Agent Orange. Agent Orange was used extensively by the U.S. military in the Korean demilitarized zone and Vietnam. The Department of Veterans Affairs recognizes that CLL can result from exposure to this toxin.

There is no screening test currently in place for CLL. Your doctor may first suspect you have CLL from routine blood test results.

People with CLL have too many lymphocytes in their blood. Lymphocytes are a type of white blood cell. Having more than 5,000 lymphocytes per cubic millimeter of blood strongly indicates CLL. Another indicator is that your blood may also contain too few red blood cells and platelets.

If CLL is suspected, your doctor will order additional diagnostic tests. These include:

• Peripheral blood smear: A sample of blood is analyzed under a microscope to look for abnormal lymphocytes, called smudge cells.
• Bone marrow aspiration and biopsy: Bone marrow samples can help determine how advanced CLL is, but they are not required to make the diagnosis. A sample of blood marrow typically is taken from the back of the hip bone. The size, shape, cellular makeup, and pattern of spread of the white blood cells found in blood marrow are analyzed.
• Cytogenetics testing: Bone marrow cells may be grown in a laboratory for this genetic test, which takes several weeks. It looks for chromosomal anomalies in CLL cells. Cells that are missing part of chromosome 17 or defects in chromosome 11 may indicate a less hopeful prognosis. Cells missing part of chromosome 13 may indicate slow-growing CLL and a more hopeful prognosis.
• Fluorescence in situ hybridization (FISH): This test looks for abnormalities in the chromosomes. It takes far less time to perform than cytogenetics (just a few days). It allows a prognostic category (good, intermediate, or poor) to be assigned.
• Flow cytometry: A flow cytometer instrument can detect CLL cells in the blood or bone marrow. Flow cytometry is also helpful for detecting zeta-chain-associated protein kinase-70 (ZAP-70) and cyclic ADP ribose hydrolase (CD38) on the surface of CLL cells. Finding many cells with these substances may indicate a less hopeful CLL prognosis.

CLL Staging

Staging can help your doctor determine CLL prognosis and spread. It can also determine which treatments will be most effective.

There are two different systems used for staging CLL. The Rai system is most typically used in the United States, while the Binet system is more commonly used throughout Europe.

The Rai system is comprised of five stages, from 0 to 4, that are grouped into three risk groups:

• Stage 0 is low risk
• Stages 1 and 2 are intermediate risk
• Stages 3 and 4 are high risk

To determine CLL stage, the Rai system takes these factors into account:

• Lymphocytosis (high lymphocyte count)
• Enlargement of the lymph nodes, spleen, and liver
• Platelet count (whether it is low, indicating thrombocytopenia)
• Red blood cell count (whether it is low, indicating anemia)
  

CLL Treatment Options

There is currently no cure for CLL. Treatments are designed to help you manage symptoms, plus slow down the progression of the disease. Your doctor may recommend one or more treatments for you based upon the stage you’re in, the symptoms you have, and other factors such as age and health. Treatments include:

Watch and wait: If you’re in a low-risk stage and have no outward symptoms, watchful waiting may be the first recommended course of action. Watchful waiting entails observation without starting treatment. It has been shown in studies to be as effective as aggressive treatments during early CLL stages. It also has the advantage of reducing the potential for side effects from treatments.

Watchful waiting is not a passive treatment. During the watch-and-wait period, you’ll see your doctor regularly for examinations and blood draws.

Chemotherapy: Chemotherapy fights cancer with drugs that are taken orally or intravenously. Chemo drugs used to treat CLL include corticosteroids, alkylating agents, and purine analogs.

Monoclonal antibodies: Monoclonal antibodies target and destroy a specific target, such as proteins on the surface of cancer cells. They are given intravenously. Several types of monoclonal antibodies are used to combat CLL. Chemotherapy, combined with monoclonal antibody treatment, is a standard protocol used for fighting this disease.

Radiation: Radiation destroys or shrinks cancer cells in a specific, pinpointed location. Since CLL doesn’t usually cause tumors, it is not a standard therapy for this condition. However, low-dose radiation may be used if you have an enlarged spleen or swollen lymph nodes in a specific area.

Targeted therapy: Targeted drugs may also be used as a first-line CLL treatment. Targeted therapy drugs zero in on proteins in and on CLL cells, to block or disrupt their activity.

Stem cell (bone marrow) transplant: This treatment may be recommended if other therapies fail, or if you have a recurrence. A stem cell transplant uses immature cells called stem cells to replace cancerous bone marrow.

Surgery: If you have an enlarged spleen that is causing symptoms such as pain, a splenectomy (removal of the spleen) may be recommended.

Is a Cure for CLL in Sight?

There is currently no cure on the horizon for CLL. Research is ongoing and continues to prolong survival and improve the quality of life for people with this condition. Cutting-edge treatments, including the use of monoclonal antibodies (mAbs, proteins made in a lab to fight infection) and targeted therapies (using drugs or other substances to identify and attack certain cancer cells), are helping people live longer and with fewer symptoms than in decades past.   

Current research into potential cures and treatments for CLL include:

• Vaccine therapy: The immune system does not recognize cancer cells as being foreign. Vaccine therapy stimulates the immune system to fight against cancer cells. CLL and its treatments can suppress the immune system. This is a challenge researchers will have to overcome for vaccine therapy to work.
• Chimeric antigen receptor T cell therapy (CAR-T): In this treatment, some of the patient's immune cells called T cells are changed so they attack specific proteins on the surface of cancer cells. These T cells are infused back into the patient.
• Gene therapy: Gene transfer therapy introduces new genes into cancer cells to slow their growth or cause cell death.

Coping and Support for Chronic Lymphocytic Leukemia

Receiving a diagnosis of CLL can be scary and upsetting. People with early-stage disease who are watching and waiting may find it stressful to not pursue treatment.

Those with later-stage disease may have uncomfortable side effects from CLL and its treatments. Dealing with an incurable illness is never easy. These strategies may help:

Speak Out

Be an advocate for your own care. If you have questions or concerns, talk to your doctor or to other members of your CLL team, such as a therapist or social worker. This may be especially important if your condition or treatment protocol changes.

Educate Yourself

You don’t want your entire life to be about CLL, but it’s good to keep on top of developments and new research. Try to find the balance. Educate yourself about your condition and what you can do to maintain a high quality of life. Remember also to leave time for other important things, including family, friends, and interests.

Stay Active

CLL may cause fatigue. The treatments you’re going through may also make you feel very tired, or ill. You’ll probably have good days and bad days. That’s to be expected.

Whenever possible, try to exercise and stay active. Taking walks, swimming at the gym, and doing easygoing activities like window shopping can help you stay engaged and social. Go at a pace that is comfortable for you. When possible, enroll a friend or exercise buddy to tag along.

Engage in Self-Care

Self-care is more than just a catchphrase. It’s a strategy that can help you find strength, enjoy life, and support health.

Self-care will vary for different people. For you, it may include pursuing meditation and yoga or eating healthy, organic food. Or, it may mean putting on makeup every day, even when you don’t feel like getting out of bed.

Try to include good sleep, exercise, and social time with people you love in your self-care plan. Making time for activities you enjoy and feel nurtured by has no downside.

Summary

CLL is a slow-growing cancer. It is possible to have this condition for many years before you start to show symptoms. The prognosis for CLL is much higher than for many other types of cancer. However, there is currently no cure for this disease. CLL has a five-year survival rate of around 83%, although it drops under 70% in those over 75.

A Word From Verywell

Finding out that you or a loved one has CLL may take your breath away. It can be overwhelming to learn you have an incurable condition. It may sound cliché, but incurable and untreatable are not the same thing.

There are many highly effective treatments that improve survival rates and quality of life for people with CLL. It’s also important to remember that CLL survival rates are higher than those for many other types of cancer.

No matter what the future brings, try not to go through it alone. Support groups for CLL patients and their caregivers can be found through the CLL Society and the Smart Patients Chronic Lymphocytic Leukemia Community.