Leukemia is a cancer of the white blood cells that arises from the bone marrow and circulates in the blood. It is characterized by uncontrolled growth of white blood cells. Leukemia is a disease of the blood cells and does not usually form a solid tumor. Although leukemia starts in the bone marrow, it can spread to the blood, lymph nodes, spleen, liver, central nervous system (CNS) and other organs.

There are four major types of leukemias:

• Acute myelogenous leukemia (AML)
• Chronic myelogenous leukemia (CML)
• Acute lymphocytic leukemia (ALL)
• Chronic lymphocytic leukemia (CLL)

The terms "myelogenous" and "lymphocytic" denote the different types of white blood cells involved. The terms "acute" or "chronic" refer to the rate of progression of the disease.

Chronic lymphocytic leukemia (CLL) represents approximately 33% of all leukemias and occurs most frequently in the elderly population. Approximately 66% of patients with CLL are 65 years of age older and the median age at the time of diagnosis is 72. Chronic lymphocytic leukemia usually does not develop in people under age 40 and is also extremely rare in children.

Chronic lymphocytic leukemia affects a particular type of white blood cells called B lymphocytes. For this reason, a more accurate name for this condition is B-cell chronic lymphocytic leukemia. Lymphocytes are specialized white blood cells of the immune system that play a major role in the body's ability to fight-off infections.

Although the exact cause of chronic lymphocytic leukemia is currently not known, researchers have recently discovered that certain mutations (genetic alterations or errors) that occur in the DNA of normal bone marrow cells can cause these cells to transform into leukemic cells.

Cytogenetic studies, which are special tests that can detect specific genetic mutations of chromosomes, have shown that patients with CLL often have a loss of part of a chromosome. This type of genetic mutation is called a deletion. In patients with CLL, the deletion is most often seen on chromosome 11 or 13. Other chromosomal abnormalities can also be detected in patients with CLL such as an extra chromosome 12 (trisomy 12).

In the early stages of the disease, clinical symptoms of chronic lymphocytic leukemia may not be obvious or may be overlooked by the patient. As the disease progresses, however, signs and symptoms become more evident. Patients may complain of generalized weakness or fatigue, may notice swollen lymph nodes, or develop recurring infections.

It is important to note, however, that approximately 50% of patients with CLL have no clinical symptoms of the disease at the time of presentation and that many cases of CLL are detected by a routine blood test in persons with no clinical symptoms. The major blood abnormality in people with CLL is an abnormal increase in the number of lymphocytes in the bloodstream, a condition known as lymphocytosis.

Chronic lymphocytic leukemia must be distinguished from other conditions that can cause similar signs and symptoms, including:

• Hairy cell leukemia
• Mantle cell leukemia
• B-cell prolymphocytic leukemia
• Leukemic phase of non-Hodgkin's lymphoma
• T-cell chronic lymphoproliferative disorders

Currently, with the possible exception of stem cell transplantation in younger patients, there is no know cure for chronic lymphocytic leukemia. Based on experience gained over the years in managing patients with CLL, doctors have developed the following general principles of treatment:

• Because CLL is a chronic disease, it is often monitored for several years without the need for any treatment intervention.

• In general, treatment is only initiated when there is clinical or laboratory evidence that the disease is progressing to a more advanced stage.

• If treatment is indicated, patients should be offered the opportunity to participate in a clinical trial.

• Patients with CLL who may benefit most from early, aggressive therapy include:

  • younger patients
  • patients in the more advanced stages of CLL
  • patients with adverse prognostic factors

• Patients with CLL are particularly at high risk for developing infections that, in some cases, may be life-threatening. The prevention and treatment of infections, therefore, is a primary goal in the management of patients with CLL.

Approximately 5% to 10% of patients with chronic lymphocytic leukemia develop a high-grade (aggressive) form of non-Hodgkin's lymphoma called a large B-cell lymphoma. This transformation of CLL to non-Hodgkin's lymphoma is known as Richter's syndrome or Richter's transformation. This disease transformation is often accompanied by symptoms such as fever, night sweats, weight loss, and enlargement of the lymph nodes, spleen, and liver. Richter's syndrome is usually treated with aggressive combination chemotherapy.

The prognosis (chance of recovery) for patients with chronic lymphocytic leukemia (CLL) can be difficult to predict with any degree of accuracy because of differences in the rate of progression of the disease between patients and individual variations in terms of response to treatment. In general, a majority of patients with CLL survive 5 to 10 years. Research has shown that, as a group, women with CLL tend to respond better to treatment and survive longer than men.

Factors that affect the prognosis for CLL patients include:

• The stage of the disease
• The extent of spread of lymphocytes within the bone marrow
• The patient's response to treatment
• Whether a patient develops serious complications such as recurrent infections or a secondary cancer
• The patient's overall health

Knowledge is Critical when Dealing with a Life-Altering Condition such as Chronic Lymphocytic Leukemia

If you or a loved one has been diagnosed with CLL, it's critical to learn everything you possibly can about this condition so that you can make informed decisions about your treatment. That's why we created the Medifocus Guidebook on Chronic Lymphocytic Leukemia, a comprehensive 182 page patient Guidebook that contains vital information about CLL that you won't find anywhere in a single source.

The Medifocus Guidebook on Chronic Lymphocytic Leukemia starts out with a detailed overview of the condition and quickly imparts fundamentally important information about CLL, including:

• The theories concerning the underlying causes of CLL.
• The specific types of genetic mutations that are found in people with CLL.
• The risk factors that can increase a person's chances for developing CLL.
• How CLL is diagnosed based on factors such as signs/symptoms, physical examination, blood tests, and specific genetic abnormalities.
• A detailed description of the two major staging systems, called Rai and Binet, that doctors use in order to:
  • Determine the extent of progression of the disease
  • Develop an optimal treatment plan
  • Predict the most likely outcome (prognosis) in terms of expected years of survival

Understanding the Standard Treatments...and the Treatment Options

Because currently there is no known cure for CLL, understanding the standard treatments - and the treatment options - is critical in attempting to prolong survival and maintain the patient's overall functional ability and quality of life. As you read through the section of the Guidebook that focuses on the treatments for CLL, you will specifically learn about:

• Which patients with CLL do not require any treatment for several years and may benefit from a "watchful waiting" approach where the disease progression is monitored carefully but no specific CLL treatment is given.
• The National Cancer Institutes guidelines for determining when initial treatment of CLL is indicated and should be started.
• The two most commonly used classes of chemotherapeutic agents that are used for the treatment of CLL, known as alkylating agents and purine nucleoside analogs.
• A new alkylating agent called bendamustine (Treanda) that was recently approved for the treatment of CLL.
• The side-effects associated with CLL chemotherapy and how to minimize their impact on your quality of life.
• A novel approach known as monoclonal antibody immunotherapy that has recently come into clinical practice which gives doctors the ability to preferentially target and destroy CLL cells.
• The role of stem cell transplantation in the management of patients with CLL, including the risks and benefits of this procedure.
• The complications, such as infection, anemia, and secondary cancers that may occur in people with CLL, including the management of these serious complications.
• The treatment options for patients with CLL who experience recurrent disease, meaning that the leukemia has come back after the patient had been in remission for a period of time.
• The role of complementary and alternative therapies in the management of patients with CLL.
• The prognosis (outlook) for people with CLL and the important factors that have a significant impact in predicting the overall chances of recovery and survival.
• Quality of life issues such as sleep disorders, fatigue, weight loss, and psychological stress that can negatively affect people with CLL and how to minimize the impact and cope better with these issues.
• Important questions to ask your doctor about CLL.

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Updated: January 17, 2023
182 Pages