Leukemia (CLL)

Chronic lymphocytic leukemia (CLL) is the most common type of the leukemia. A leukemia is a cancer the begins in the blood-forming cells of the bone marrow. When the cells become leukemic, they stop maturing properly and grow out of control. Eventually they spill into the blood stream. CLL accounts for about 25% of new cases of leukemia every year.

Because CLL is a disease that develops slowly, many people live for years without any symptoms. The first signs of CLL are often detected using blood work during a routine doctor's visit. Several blood tests will be done to confirm the diagnosis. Someone with CLL usually has a high white blood cell count and a low red cell count.

Some people may have physical symptoms — like weight loss, fatigue, or swollen lymph nodes — however, often symptoms aren’t evident until CLL has advanced.

Symptoms & Diagnosis

What are Blood Cancers?

Leukemias are cancers that start in the blood-forming cells of the bone marrow. When these cells become leukemic, they stop maturing properly and grow out of control. Eventually, they spill into the bloodstream and prevent your blood from doing normal processes like fighting infections, keeping your energy up and preventing excessive bleeding.

  • Chronic lymphocytic leukemia (CLL) is the most common type of leukemia that affects older adults
  • The average age of most patients at diagnosis is about 70 years old
  • It is rare for CLL to occur in people under age 40, and it is even more rare in children
  • CLL accounts for about one-quarter of new cases of leukemia each year
  • Because it is a disease that develops slowly, many people live for years without symptoms

Causes of CLL

No one knows exactly what causes chronic lymphocytic leukemia (CLL). CLL develops from de novo mutations, or new genetic mutations, that scientists don't yet fully understand. Potential causes include:

  • Genetic mutation in the DNA
  • Exposure to something in the environment, like chemicals or radiation
  • Testing For CLL

The first signs of chronic lymphocytic leukemia (CLL) are often detected by blood work during a routine doctor's visit.

  • Flow cytometry test identify the distinctive markers that distinguish CLL cells
  • Imaging tests or biopsies are not a standard part of the initial workup, unless your doctor is concerned about specific symptoms like extremely enlarged lymph nodes
  • Blood Count and CLL

Once the CLL diagnosis is determined, doctors focus on the blood count to gauge how advanced the disease is and when treatment is necessary. Specifically they look at the abnormal increase in the number of lymphocytes (a type of white blood cells)  in the blood and marrow. If you get a diagnosis of CLL, it's a good idea to get a second opinion.

Categories of Blood Cancer

Leukemias are broken down into different categories based on the cells that grow into cancer cells and how quickly those cells grew. There are four basic categories doctors use to identify the different types of leukemia.

  • Acute leukemia grows very quickly
  • Chronic leukemia grows slower, over several years
  • Lymphoid leukemia grows from lymphoid cells, which produce antibodies and protect against viruses
  • Myeloid leukemia grows from myeloid cells, which is the body's first defense for bacteria

Tests that Inform the Diagnosis

  • Fluorescent in situ hybridization (FISH) is the first line of genetic or molecular testing is often looking at the chromosome amplifications or deletions that are unique to your cancer. The information from this test can help give a ballpark idea of the aggressiveness of your cancer, as well as the general life expectancy for people who have similar chromosome abnormalities.
  • Immunoglobulin heavy chain variable region (IGHV) is a test that looks at the gene for producing the immunoglobulins or antibodies that help you fight infections. This test checks if the genetic instructions for the immunoglobulin heavy chain variable region (IGHV) have changed to make your CLL more or less aggressive.

Monitoring CLL After Diagnosis

In chronic lymphocytic leukemia (CLL), not all patients need treatment at diagnosis. Only about a third of patients do. During the early stages of the disease, people are monitored and get blood tests every few months in an active surveillance approach. But if their blood count is stable over several years, monitoring becomes less regular.

IGHV Mutation Status

Doctors looking for a patient's IGHV mutation status are looking at the age of the cell that allowed CLL to grow. If it is a younger, unmutated cell, it will likely be more aggressive. If it is an older, mutated cell, those tend to be slow-growing.

  • Typically, a patient with a mutated IGHV cell will go into remission for 10 to 15 years after initial treatment
  • A patient with an unmutated cell will typically only have 3 to 4 years of remission

Treatment

Stages of CLL

Doctors use the Rai staging system for chronic lymphocytic leukemia (CLL) which involves only blood tests and a physical exam. The stages are broken up into early-stage (stages zero, 1 and 2) and advanced (stages 3 and 4). Early in the disease, the lymphocyte count is high and the lymph nodes, liver and spleen may be effected to varying degrees.

  • Early-stage patients usually do not require any therapy and may be placed on observation for months, or even years
  • Advanced-stage means the disease has infiltrated the bone marrow, and caused low red blood cell count (anemia) or low platelets (thrombocytopenia)

When Treatment is Needed

CLL patients are monitored and their blood count is tracked over time. The blood counts are followed because they act as a marker for how extensively the bone marrow is involved with disease. And if the marrow has significant enough involvement that may lead to CLL treatment.

New Approach to Treatment

New drugs that have been approved for CLL treatment in the past couple of years have drastically changed the way the disease is treated.

  • CLL treatment in the past consisted of intravenous (IV) medication
  • New oral medications for CLL treatment have improved survival rates
  • The downside of the oral medication is that patients have to commit to taking them every day for the rest of their lives
  • Doctors are now combining some of these oral agents with even some gentler IV treatments, and are researching combinations of oral agents without using any IV treatments

Initial Treatment Depends on Age

Standard treatment will be some form of chemo-immunotherapy. A chemo-immunotherapy regimen is given as initial therapy for six months at a time — any treatment lasting longer than six months is difficult to tolerate. The exact regimen depends on the patients age.

  • For patients under age 65, the go-to chemo-immunotherapy regimen is usually FCRa combination of Fludarabine, Cyclophosphamide and Rituximab. Almost all patients will go into remission after this treatment, and about 50 percent of those patients will go into complete remission.
  • For older patients, doctors may use a different chemo-immunotherapy treatment called BR, which is often a combination of Bendamustine and Rituximab.
  • For patients who may even be older or too frail for BR, another treatment option is combining the oral chemo drug chlorambucil with the CD20 antibody obinutuzumab.

Oral Medicine for Relapsed CLL: Venetoclax

Venetoclax is an option for patients with relapsed chronic lymphocytic leukemia (CLL). Venetoclax is

  • a highly-potent drug that can kill CLL cells quickly
  • a Bcl-2 inhibitormeaning it targets the Bcl-2 protein on CLL cells

CLL Venetoclax treatment can be very successful in treating patients with relapsed CLL, but careful monitoring is necessary. One of the known side effects of Venetoclax is something called tumor lysis syndrome, which is when a tumor breaks open too quickly and releases toxins into the blood stream. This could be dangerous for the kidneys and the heart, so patients taking Venetoclax will need to be assessed for their risk of developing

Oral Medicine for CLL: Ibrutinib

Ibrutinib is among the new oral medications approved for treatment of CLL. It is a BTK inhibitormeaning it targets abnormal cells. Common side effects of the drug include:

  • increased risk of bleeding or bruising
  • atrial fibrillation (rapid heart rate)
  • diarrhea
  • joint aches
  • rash
  • high blood pressure
  • Most patients experience one or two side effects, and some experience none.

Ibrutinib is a chronic medication, so patients could be on it for years and may find it hard to take a daily pill for so long

Oral Medicine for People With Relapsed CLL: Idelalisib

Idelalisib is an oral medication that's FDA-approved for patients with relapsed CLLand it's given in combination with rituximab and is a generally very effective drug. Side effects of the drug that could be cause for concern include:

  • Diarrhea, which can lead to colitis, as well
  • Liver irritation
  • Lung inflammation

But these side effects are rare and the treatment has a high success rate.

Idelalisib is not available for previously untreated patients because trials in that setting showed increased death rates due to infection. All trials exploring idelalisib in the frontline setting were shut down. The increased rates of death due to infection were not seen in trials done in the relapsed setting.

Monoclonal Antibodies and CLL

Monoclonal Antibodies have been used as CLL treatment for at least twenty years. The medication targets a protein on CLL cells called CD20.

  • Doctors will start patients off with a pre-medication to get their bodies used to the drugs
  • Up to 50% percent of those patients may experience a reaction on the initial exposure to the transfusion that could include a change in blood pressure and/or fever or chills
  • In subsequent transfusions however, that reaction rate drops to 10% or less

There are two drugs in this class commonly used for CLL treatment

  • Rituximab is the oldest drug in this class and is used widely in CLL treatment. When it is combined with chemotherapy, it enhances the ability to kill CLL cells
  • Obinutuzumab was approved more recently as an initial therapy for CLL. it's proving to be even more potent than rituximab in clinical trials and is leading to longer remissions

Later Stage Treatment

Clinical Trials Test State-of-the-Art Cancer Treatments

New treatment options allow patients with chronic lymphocytic leukemia (CLL) to live longer than they used to with the former standard of care, chlorambucil-based chemotherapy. Clinical trials are underway for a variety of options:

  • Chemo-immunotherapy
  • Monoclonal antibodies that enhance the immune system
  • Small molecule targeted therapies that take advantage of specific weaknesses in cancer cells

Through clinical trials, doctors are able to carefully study and learn which new regimens are better at treating CLL. They're also one of the best ways for patients to get state-of-the-art cancer treatment.

Stem Cell Transplant for CLL

Stem cell transplants can be a significant part of treatment for blood cancers but it's not widely used for CLL. Stem Cell Transplant is a procedure that replaces your unhealthy blood-forming cells with healthy cells. This helps eliminate the cancer from your bone marrow and restore normal blood cell function.

  • It's not used very often anymore in treating CLL for a number of reason including the fact that it's not curative and also because of the advent of new oral drugs.
  • The treatment is sometimes an option for patients who are no longer responding to medication.

New Treatments for Relapsed CLL Patients

A new study out from an Oregon hospital shows that combining an oral tablet called idelalisib (also known as Idela) with rituximab, which had traditionally been prescribed for use with chemotherapy, is both a safe and effective treatment for this chronic disease.

Symptoms at Advanced Stages of the Disease

Physical symptoms are rare at the time of diagnosis, but often occur at much later stages of the disease. People who do start to show some of these symptoms may develop:

  • Swollen lymph glands
  • Enlarged spleen

People with CLL may also feel systemic symptoms including:

  • Fatigue
  • Unintended weight loss
  • Drenching night sweats
  • Frequent infections

Richter’s Transformation

In about ten percent of CLL patients, their disease transforms into a more aggressive lymphoma called Richter's Transformation, which it is much more difficult to treat. Patients who suffer from Richter's Transformation may experience symptoms including:

  • Fever
  • Night sweats
  • Weight loss

Clinical Trials Using CAR-T Cells

An exciting area of development when it comes to CLL treatment is CAR-T cells or chimeric antigen receptor T-cells. CAR-T therapy involves removing T-cells from the body and training them to recognize cancer cells in a laboratory.

CAR-T cells have shown promise for treating lymphoma, leukemia, and myeloma. Clinical trials currently in progress are using a new kind of CAR-T cells, which are grown in a unique way that doctors hope will be better at attacking cancer cells. In order to be eligible for these trials, patients must be healthy enough to undergo therapy that can be intensive, but also sick enough to need this kind of experimental treatment. The ideal patient for this type of trial is someone who has not responded to any other treatments.

Why Choose A Clinical Trial

Clinical trials are incredibly important both from an individual and a societal standpoint. These studies give patients, who have diseases that are not responding to typical treatments, access to a bevy of new drugs that are currently being developed by pharmaceutical companies. Testing these treatments on people in experimental trials is really the best way for research to progress.

A lot of the therapies being tested in clinical trials will fail. However, participants in clinical trials are statistically more likely to have better outcomes.

Why Do People Get Excluded From Clinical Trials?

A clinical trial is essentially an experiment with willing participants, and doctors need to be able to interpret the data they receive from these trials. This can be very frustrating for patients. But the logic behind the exclusion criteria is that if a patient has extremely advanced cancer and may not make it until the end of the trial, the data could be compromised.

Clinical Trials and Statistics

“Progression-free survival” and “overall survival” are how results are measured during clinical trials.

It's important to understand the distinction between these two types of survival statistics. Improvement in overall survival means the study shows that you actually live longer because of the treatment. Progression-free survival means that you don't live longer, but you have more time when your cancer does not get any worse.

Living With Cancer

Is Green Tea Effective In Treating CLL?

Green tea has long been celebrated for its antioxidant properties but with regards to cancer, there is no proof it is effective in treating the disease.

Sugar, The Western Diet And Cancer Prevention

Obesity has been linked to several cancers as well. And since our Western diet has led to our current obesity epidemic, it's a good idea to maintain a diet that incorporates more fruits and vegetables, whole grains, and lean proteins, and less sodium, saturated fat, and added sugar.

Sugar itself has attracted a lot of attention for a possible role in cancer, but overwhelming medical evidence concludes sugar does not cause cancer.

The Difference Between Integrative Medicine and "Alternative medicine"

Many people at least consider treatments, or foods, or techniques, that come from outside the oncologist's office. A lot of cancer doctors actually support integrating other methods into their care. But there is a big difference between "integrative," or holistic medicine, and "alternative medicine," which seeks to replace approved treatments, and often has little or no serious science to support it.

CLL Will Likely Recur & Need Further Treatment

Treatment of chronic lymphocytic leukemia (CLL) is not considered to be curative. This means that after your initial round of CLL treatment through chemotherapy, there may be no signs of leukemia for years, but the cancer will probably return at some point. The first sign this is occurring again may include:

  • White blood cell count becomes elevated again, even though patient feels fine
  • Lymph nodes begin to swell again

Doctors don't usually order routine CAT scans for patients who have had initial therapy. So it's often the patients that bring their CLL recurrence to the attention of their doctor. Usually patients can do well with simple monitoring for several monthssometimes even years in case resuming CLL treatment is necessary.

Younger patients or those with more aggressive CLL may be considered for bone marrow transplants. But new targeted drugs, immunotherapies and other treatment combinations are making transplants less common for CLL.

The Doctor-Patient Relationship

The doctor-patient relationship is extremely important after a cancer diagnosis. You'll want to make sure you are working with a doctor who:

  • You trust
  • Will respect your opinion
  • Keeps you informed about all of the treatment options for your particular diagnosis
  • Is board-certified in treating cancer
  • Has seen and treated a lot of people with your cancer

Palliative Care

Palliative care is not just for making people comfortable at the end of their lives; it can be brought in for people who have both curable and incurable diseases. Palliative care is a team of health care professionals focused on:

  • Symptoms management
  • Patient comfort

Paying for Treatment; Getting Help With Cancer Bills

It's rare for health insurance to pay for 100 percent of your cancer treatment. It's something most people struggle with. However, there are resources that you may not know about.

  • It's common for doctors to  get on the phone and advocate on your behalf with your insurance company
  • Many drug companies and clinical trials also offer patient assistance programs
  • The social workers at your cancer care clinic can help guide you through the various options
  • Patient advocacy groups are also willing to help in several ways
  • Your church or social group may be another source of support

What's Mindfulness? And Can It Help You During Your Cancer Journey?

Often after a cancer diagnosis emotions of fear, anxiety, and confusion arise, along with many unanswered questions. Mindfulness is often suggested for cancer patients to reduce high levels of anxiety and distress associated with diagnosis. Here are 5 tips for practicing mindfulness during cancer treatment:

  • Choose one daily activity to practice mindfully (e.g. eating your lunch, brushing your teeth or taking a shower). During this activity notice your breath and activity of your mind for a few moments.
  • Take a pause throughout your day. During your day, find a moment to stop and take 5 deep breaths with your eyes closed.
  • Kindly acknowledge a moment you're experiencing a difficulty by putting your hand on your heart and saying, "I feel my pain. How can I be kind to myself in this moment?"
  • Get curious about your emotions.  Experiment with welcoming your emotions as they come, instead of pushing them away.
  • Become aware when you're in a rush. Ask yourself, "Do I really need to hurry?"

 

CLL

Treating CLL

SEE MORE   

CLL generally grows very slowly. So if your unhealthy white blood cells — the leukemia cells — don't outnumber or impact the rest of your blood count, there's no urgency to treat your condition. Some patients can be monitored for years and never need therapy. In fact, about a quarter of people with this type of CLL never need treatment. For those who do require treatment, some form of chemo-immunotherapy combination is usually the go-to treatment.

Treating CLL

CLL generally grows very slowly. So if your unhealthy white blood cells — the leukemia cells — don’t outnumber or impact the rest of your blood count, there’s no urgency to treat your condition. Some patients can be monitored for years and never need therapy. In fact, about a quarter of people with this type of CLL never need treatment. For those who do require treatment, some form of chemo-immunotherapy combination is usually the go-to treatment.

Diet

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There has been some evidence to suggest that the Western diet – which is high in processed meat, refined grains, sugar, and caloric drinks — can increase a person’s risk of developing CLL. However, when it comes to preventing CLL, or any cancer, common sense dieting is key.

Diet

There has been some evidence to suggest that the Western diet – which is high in processed meat, refined grains, sugar, and caloric drinks — can increase a person’s risk of developing CLL. However, when it comes to preventing CLL, or any cancer, common sense dieting is key.

CAR-T Therapy

SEE MORE   

An exciting new development when it comes to CLL treatment is CAR-T therapy — which involves removing T-cells from the body and training them to recognize cancer cells in a laboratory. This is done by genetically modifying the cells, and then growing them in large numbers. When infused back into the body, these new cells seek out and destroy the CLL cells. `

CAR-T Therapy

An exciting new development when it comes to CLL treatment is CAR-T therapy — which involves removing T-cells from the body and training them to recognize cancer cells in a laboratory. This is done by genetically modifying the cells, and then growing them in large numbers. When infused back into the body, these new cells seek out and destroy the CLL cells. `

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