Leukemia is an umbrella term for cancers of the blood and the blood-forming tissues of the body. Chronic lymphocytic leukemia (CLL) is the most common type of the disease. Most cases are in people age 55 and older.
Like all types of leukemia, CLL starts with problems in the creation of blood cells.
Blood cell creation
The body produces millions of blood cells each day. Most develop in the bone marrow, the spongy interior of bones that contains immature stem cells.?
In a healthy person, these immature stem cells first become either lymphoid stem cells or myeloid stem cells.
Lymphoid stem cells develop into white blood cells, which are immune system cells. They start by becoming immature white blood cells known as lymphoblasts, then mature into lymphocytes. The two types of lymphocytes that are usually involved in leukemia are B cells and T cells. B cells produce the antibodies responsible for attacking bacteria and viruses that invade the body. T cells help alert other immune cells to the presence of infection or fight infection directly.
Myeloid stem cells also develop into white blood cells. The myeloid stem cells first become immature white blood cells known as myeloblasts. They then mature into monocytes and granulocytes, including neutrophils, all of which fight disease. Other myeloid stem cells develop into red blood cells, which carry oxygen throughout the body; and platelets, which help the blood clot.
Leukemia occurs when the DNA (the genetic instructions that control cell activity) of a bone marrow stem cell mutates at some point in its development. The cell becomes cancerous, begins multiplying rapidly and crowds out healthy cells in the blood and bone marrow. These diseased cells can also gather in specific parts of the body, including the liver, lymph nodes, spleen and skin.
How is leukemia classified?
While there are many types of leukemia, they are typically classified by the type of stem cell that has turned cancerous, either lymphoid or myeloid.
Many types are also classified as either chronic or acute. Acute leukemia?impacts immature cells, preventing them from developing and carrying out their function. These cells tend to multiply rapidly, making acute leukemia more aggressive.?
Chronic leukemia?involves mature or partially mature cells. These cells multiply more slowly and are less aggressive, making chronic leukemia less aggressive than acute leukemia.
About CLL
CLL develops from B cells. B cells start out as lymphoid stem cells, then mature into adult B cells, which help fight disease. In CLL, a cancerous B cell grows and multiplies in the bone marrow, lymph nodes, liver and spleen, resulting in a high white blood cell count. These cancerous cells are not able to fight infection. They also crowd out healthy cells from the marrow and cause enlargement of lymph nodes, liver and spleen.
More than 20,000 people are diagnosed with CLL in the U.S. each year. The disease is virtually unheard of in children, and it primarily affects older adults. Almost 90% of new cases are in people age 55 and older, and the average age at the time of diagnosis is around 70.
As a chronic disease, CLL is less aggressive than acute forms of leukemia. It has a five-year survival rate of around 87%. The presence of specific genetic mutations and proteins produced by the diseased cells, however, can have a big impact on the patient¡¯s prognosis.
CLL risk factors
A risk factor is anything that increases the chance of developing a disease. Knowing a disease¡¯s risk factors can be an important step towards catching it early. It's important to note that not everyone with risk factors will develop the disease.
Risk factors for CLL include:
- Family history: People with a parent, sibling or child who has had CLL are more likely to develop the disease.
- Age: Almost 90% of new CLL cases are diagnosed in people age 55 and older.
- Sex: More men develop CLL than women.
- CLL is more common among white and Black people, while it is very rare among people of Asian descent.
- Agent Orange: Exposure to this chemical is a known risk factor for CLL.
Learn more about chronic lymphocytic leukemia:
- Chronic lymphocytic leukemia symptoms
- Chronic lymphocytic leukemia diagnosis
- Chronic lymphocytic leukemia treatment
Learn more about clinical trials for chronic lymphocytic leukemia.
Blood cell creation (click to enlarge).
Leukemia specialists: How advances in measurable residual disease (MRD) are improving cancer care
Measurable residual disease (MRD) is a monitoring tool that physicians use to assess the remission status of patients with leukemias, lymphomas, myelomas and other blood disorders.??
Formerly known as ¡°minimal residual disease,¡± this term describes a situation in which cancer cells are still present in the body after treatment, yet undetectable using current testing methods.?
Two of our leukemia specialists, , and , explored this subject in a recent . Here are three highlights from that discussion.?
Next-generation sequencing can now reveal ¡®a needle in a haystack¡¯
Patients with blood cancers used to be monitored for relapse using:?
- Flow cytometry, which reveals disease through certain patterns of refracted laser light, and?
- Polymerase chain reaction (PCR) tests, which are highly sensitive molecular tests that detect specific genetic material from cancer cells?
But sometimes, patients would seem to be cured based on these tests, only to relapse some months later. That got doctors to questioning why. Here¡¯s the conclusion ours came to: even if some patients appeared to have no residual disease, it was only because we weren¡¯t able to detect it yet.??
One possible solution? Next-generation sequencing.?
¡°With next-generation sequencing, we can pick out a single leukemia cell from a sea of a million normal ones,¡± explains Haddad. ¡°This is a very, very minimal level of disease that would¡¯ve previously gone unnoticed ¡ª like a needle in a haystack. This new test can help us detect any little seed left behind so we can completely eradicate the cancer. This marks a real paradigm shift.¡±?
Richter transformation: What chronic lymphocytic leukemia (CLL) patients should know
Richter transformation, historically called Richter syndrome, can affect patients who have or have not been treated for chronic lymphocytic leukemia (CLL). In rare cases, it can occur in previously untreated patients with CLL.?
Named after Maurice Richter, Ph.D., Richter transformation occurs when chronic lymphocytic leukemia changes into a more aggressive disease ¨C usually diffuse large B-cell lymphoma or, much less commonly, Hodgkin lymphoma.?
We spoke with CLL specialist William Wierda, M.D.,?Ph.D., to learn more about Richter transformation.?
What are the symptoms of Richter transformation??
Patients usually experience the following symptoms simultaneously:
- Fever
- Rapidly enlarging lymph nodes
- Electrolyte abnormalities
- Fatigue
Keep in mind that the syndrome is rare. ¡°Less than 10% to 20% of patients develop Richter transformation over the course of their disease,¡± says Wierda.
How is Richter transformation diagnosed?
If a chronic lymphocytic leukemia patient experiences symptoms of Richter transformation, their oncologist will order a PET-CT scan to look for changes to the size and metabolic activity of lymph nodes.
¡°In CLL, we can see large lymph nodes, but none of the lymph nodes will show high metabolic activity, reflected by a high SUV on the PET-CT scan,¡± explains Wierda. ¡°In Richter, there is more metabolic activity in the lymph nodes, which can be seen on these scans and is used to direct the location for lymph node biopsy required to document the disease.¡± ?
Leukemia cells circulate in the blood, a simple blood draw can tell your doctor more information when they¡¯re making a CLL diagnosis. If your doctor thinks you might have Richter transformation, they¡¯ll biopsy lymph nodes. If the biopsy shows cells that are large and active, this indicates aggressive lymphoma.?
What causes Richter transformation?
¡°The cause of Richter transformation is still not fully understood,¡± says Wierda. ¡°Some patients have an increased risk of developing it if they have a mutated gene called NOTCH1 or they have multiple chromosomal abnormalities in the leukemia cells.¡±
Can the transformed disease be treated??
By the time they¡¯re diagnosed with Richter transformation, many patients have already undergone prior treatment for CLL. For these patients, chemotherapy does not work as well to treat the transformed disease. ?
¡°´¡³Ù MD Anderson, the treatments we give for patients who have undergone Richter transformation are sometimes like standard lymphoma treatment. But we have a lot of clinical trials going on to explore other treatment options,¡± says Wierda.?
Stem cell transplants have shown long-term results for Richter transformation.
¡°Our clinical trials are looking at the promise of CAR T cell therapy and a bispecific monoclonal antibody treatment,¡± says Wierda. ¡°These trials are very important. Understanding Richter transformation is one of the biggest unmet needs for patients with CLL.¡±
Why is it important to go to a research hospital like MD Anderson for treatment for Richter transformation??
¡°Richter transformation can be challenging to treat,¡± says Wierda. ¡°Because there is no standard treatment, patients who come to MD Anderson for treatment can access treatment options through research initiatives and clinical trials that may not otherwise be available to them.¡±
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Why choose MD Anderson for your leukemia treatment?
Choosing the right cancer center may be the most important decision you can make as a leukemia patient. At MD Anderson¡¯s Leukemia Center and Stem Cell Transplantation and Cellular Therapy Center, you¡¯ll get treatment from one of nation¡¯s the largest, most experienced leukemia teams at a top-ranked cancer center.
Using a comprehensive team approach, we work together to give you customized care that includes the most advanced diagnostic methods and treatments. These include clinical trials of new drugs and drug combinations. We offer clinical trials for all situations ¨C from patients receiving their first treatment, to patients who have exhausted all standard treatment options.
As a leading center for leukemia care, we offer access to innovative new therapies and clinical trials that may help increase your chances for successful treatment. Many of these were developed by our own researchers.
Treatment designed specifically for you
Successful leukemia treatment begins with?accurate?and precise diagnosis. Many of our leukemia patients have been misdiagnosed before they come to MD Anderson. We have the expertise and experience gained from being one of the most active programs in the world, and our specialized pathologists are highly skilled in diagnosing leukemia.
Our approach to leukemia is customized especially for you. We carefully evaluate your risk factors and the specific characteristics of your leukemia to determine if immediate treatment is necessary. If it is, we recommend the most effective therapies while aiming to limit treatment side effects.
Whether you are treated as an inpatient or outpatient, our comprehensive program offers all the services needed to care for leukemia and respond to its impact on your?body. We aim to accomplish as much care as possible on an outpatient basis. If hospitalization is needed, our expert staff is specially trained to care for patients at every phase of the treatment journey.
MD Anderson is my hope. I know that without it, I wouldn¡¯t be alive today.
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