Learn about chronic lymphocytic leukemia (CLL), including its prevalence, causes, treatment options, and survival rate. Get the latest information on CLL at Meri Seat.

Chronic Lymphocytic Leukemia

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Syed Intisar Hussain

Overview

Chronic lymphocytic leukemia (CLL) is a type of cancer that affects the blood and bone marrow. 
&nbsp;

Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in adults in Western countries, accounting for approximately one-quarter of all new leukemia cases<a href="#accordion">[2]</a>. It primarily affects older adults, with the average age of diagnosis being around 70 years old<a href="#accordion">[2]</a>. According to the American Cancer Society, about 18,740&nbsp; new cases of CLL will be diagnosed in the United States in 2023, and approximately 4,490 people will die from the disease<a href="#accordion">[2]</a>.

Chronic lymphocytic leukemia is characterized by the bone marrow making too many abnormal lymphocytes. An accumulation of these abnormal cells can cause a wide variety of symptoms, such as anemia and easy bleeding. The disease may progress slowly or very fast.

The incidence of CLL has been increasing over the past few decades, which may be due in part to the aging of the population. It is more common in men than women and is more prevalent in certain ethnic groups, including those of Eastern European Jewish descent<a href="#accordion">[4]</a>. Asian populations rarely report cases of CLL. 
&nbsp;

According to research, there are two main types of chronic lymphocytic leukemia (CLL) - slow-growing and fast-growing. 
&nbsp;

<ul>
	<li>Slow-Growing CLL: This is characterized by an increased number of lymphocytes but normal red blood cell, neutrophil, and platelet levels. Since it grows very slowly, the patient may remain stable for years before requiring treatment.</li>
	<li>Fast-Growing CLL: There is a high number of abnormal lymphocytes in the blood which affect the production of normal red blood cells and platelets. This is a more severe disease. 
	&nbsp;</li>
</ul>

Chronic lymphocytic leukemia can also be categorized based on the type of cells affected<a href="#accordion">[5]</a>.

<ul>
	<li>B-Cell CLL: This accounts for more than 95% of cases. In this form of CLL, the B-lymphocytes which are responsible for producing antibodies are affected.</li>
	<li>T-Cell CLL: T-lymphocytes control the body&rsquo;s immune response. They attack and destroy any foreign invaders or cancer cells. Approximately 1% of people with CLL have the T-cell type. T-cell CLL is called T-cell prolymphocytic leukemia. 
	&nbsp;</li>
</ul>

Signs and Symptoms

The signs and symptoms of CLL can vary from person to person and may be similar to other conditions. Some people may not experience any symptoms at all, and the disease may be detected during routine blood tests. 
&nbsp;

The following are the common signs and symptoms of chronic lymphocytic leukemia:

<ol>
	<li>Enlarged lymph nodes, especially in the neck, armpits, and groin</li>
	<li>Fatigue</li>
	<li>Fever and night sweat</li>
	<li>Weight loss</li>
	<li>Frequent infections</li>
	<li>Easy bruising or bleeding</li>
	<li>Pain or fullness in the abdomen 
	&nbsp;</li>
</ol>

It is important to note that these symptoms can be caused by many other conditions and may not necessarily indicate the presence of CLL.

Note: If you are experiencing any of these symptoms, it is important to talk to your healthcare provider for proper evaluation and diagnosis. 
&nbsp;

Causes

The exact mechanism of action of CLL is not fully understood, but it is thought to be related to the accumulation and proliferation of abnormal B lymphocytes in the bone marrow, lymph nodes, and other organs of the body. These abnormal B lymphocytes are unable to function properly and can interfere with the production and function of normal blood cells, leading to a range of symptoms and complications.

According to researchers, the pathogenesis of CLL involves a complex interplay between genetic, epigenetic, and microenvironmental factors. Abnormalities in genes such as TP53, ATM, NOTCH1, and SF3B1 have been identified as playing a role in the development and progression of CLL. 
&nbsp;

There are currently no widely accepted screening tests for chronic lymphocytic leukemia (CLL). The disease is often detected during routine blood tests or physical exams when there are no symptoms present. 
&nbsp;

Diagnosis

Chronic lymphocytic leukemia (CLL) is often diagnosed using a combination of clinical assessment, laboratory testing, and bone marrow analysis. The following tests may be used to diagnose CLL, in accordance with recommendations from the National Comprehensive Cancer Network (NCCN)<a href="#accordion">[8]</a>: 
&nbsp;

A Complete Blood Count (CBC): It is a test that counts and classifies all of the body&#39;s blood cells, including lymphocytes, which can be overrepresented in CLL.

Peripheral Blood Smear: In this test, blood cells, including aberrant lymphocytes, are examined under a microscope to determine their size, shape, and appearance.

Flow Cytometry: This test counts the number of aberrant lymphocytes as well as other particular cell types in a blood sample by using antibodies to identify and count them.

Immunohistochemistry: Using antibodies to recognize certain proteins on cell surfaces, this test can assist locate abnormal lymphocytes in tissue samples.

Bone Marrow Biopsy: To check for the existence of leukemia cells, a tiny sample of bone marrow is taken from the hip bone during this procedure. The sample is then examined for abnormal lymphocytes.

Imaging Tests: Imaging procedures including computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans can be performed to assess the severity of the condition and spot organs or lymph nodes that have enlarged. 
&nbsp;

It is essential to remember that the particular diagnostic procedures performed may rely on patient characteristics and professional judgment. 
&nbsp;

Chronic lymphocytic leukemia (CLL) is typically classified into stages based on the extent of the disease and the presence of symptoms. The two most commonly used staging systems for CLL are the Rai system and the Binet system<a href="#accordion">[8]</a>: 
&nbsp;

<h5>Rai Staging System:</h5>

The Rai system is based on five stages and takes into account lymphocytosis (high number of lymphocytes), anemia, and thrombocytopenia (low platelet count)6. It classifies patients as low-, intermediate-, or high-risk groups. 
&nbsp;

<ul>
	<li>Low Risk - There is a high lymphocyte count in the blood and bone marrow only. It was formally called stage 0.</li>
	<li>Intermediate Risk - This was formerly called stage I and II. It is characterized by high lymphocyte count, enlarged lymph nodes, splenomegaly and/or hepatomegaly.</li>
	<li>High Risk - This is characterized by lymphocytosis with anemia or thrombocytopenia. It was formerly called stages III and IV. 
	&nbsp;</li>
</ul>

<h5>Binet Staging System:</h5>

The Binet system is based on three stages and takes into account the number of enlarged lymph node groups and the presence of anemia and thrombocytopenia. 
&nbsp;

<ul>
	<li>Stage A: Fewer than three enlarged lymph node groups, hemoglobin levels of 10 g/dL or higher, and platelet level of 100 x 109/L or higher.</li>
	<li>Stage B: Three or more enlarged lymph node groups with hemoglobin and platelet levels as stage A.</li>
	<li>Stage C: This is characterized by anemia and/or thrombocytopenia. Enlarged lymph nodes may be present in any number of areas.&nbsp; 
	&nbsp;</li>
</ul>

Treatment

The degree of the disease, the patient&#39;s age, and other health-related issues all affect how chronic lymphocytic leukemia (CLL) is treated. Patients with early-stage, asymptomatic CLL may not require treatment since the condition may progress slowly or not at all. To manage symptoms and control the illness, however, medication may be required for people with advanced-stage or symptomatic CLL. Guidelines from the National Comprehensive Cancer Network (NCCN) provide a number of CLL treatment options, which may include<a href="#accordion">[8]</a>: 
&nbsp;

Watchful Waiting: This strategy entails monitoring the illness with routine physicals and blood testing, but delaying treatment until symptoms appear or the illness worsens.

Chemotherapy: It is a form of cancer treatment that employs chemicals to eradicate cancer cells. Fludarabine, cyclophosphamide, and bendamustine are the most often prescribed chemotherapeutic medications for CLL.

Targeted Therapy: Drugs are used in targeted therapy to target particular proteins or processes that contribute to the development of cancer. Ibrutinib, idelalisib, and venetoclax are the most often prescribed targeted treatment medications for CLL.

Immunotherapy: This is an approach to treating cancer that makes use of the immune system of the patient to combat the disease. Rituximab and obinutuzumab are two of the most often used immunotherapy medications for CLL.

Stem Cell Transplant: Patients with advanced-stage CLL who have not responded to conventional therapies may benefit from a stem cell transplant, commonly referred to as a bone marrow transplant. In this operation, healthy donor stem cells are used to replace the patient&#39;s damaged bone marrow. 
&nbsp;

A hematologist or oncologist should be consulted before choosing a specific course of treatment for CLL since it may rely on characteristics unique to the patient. 
&nbsp;

There are a variety of problems that can arise from chronic lymphocytic leukemia (CLL), both from the illness itself and from therapy. The following are some common complications of CLL.&nbsp;&nbsp; 
&nbsp;

Infections: CLL patients may be more prone to infections because of their compromised immune systems. <a href="https://merisehat.pk/disease/pneumonia">Pneumonia</a>, <a href="https://merisehat.pk/disease/bronchitis-acute-and-chronic-">bronchitis</a>, and urinary tract infections are examples of typical infections.

Autoimmune Conditions: Some CLL patients may develop autoimmune conditions, in which the body&#39;s immune system assaults healthy cells and tissues. Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia are a few examples of autoimmune conditions that can develop in persons with CLL

Transformation Into A More Aggressive Form Of Leukemia: In a few instances, CLL can transform into a more aggressive form of non-Hodgkin lymphoma (NHL) called diffuse large B-cell lymphoma or to Hodgkin lymphoma.&nbsp; In rare instances, CLL may progress to prolymphocytic leukemia or acute lymphocytic leukemia (ALL).

Blood Clotting Conditions: Individuals with CLL are more likely to experience pulmonary embolism and <a href="https://merisehat.pk/disease/deep-vein-thrombosis">deep vein thrombosis</a>, two conditions that cause blood to clot abnormally.

Increased Risk Of Other Cancers: People with CLL may have an increased risk of certain cancers, such as<a href="https://merisehat.pk/disease/lung-cancer-67"> lung cancer</a>, skin cancer, and gastrointestinal cancers.&nbsp;&nbsp;

Complications From Treatment: Chemotherapy and radiation therapy used to treat CLL can have a number of unfavorable consequences, including exhaustion, nausea, vomiting, hair loss, and lethargy. Treatment might occasionally result in long-term side effects including secondary malignancies. 
&nbsp;

People with CLL must collaborate closely with their medical team to control and track any potential side effects. 
&nbsp;

The following factors may increase the risk of CLL

1. Age: CLL is more prevalent in older persons, with most instances being diagnosed in patients over 60.

2. Family History: Those who have a history of CLL or other blood malignancies at a higher chance of contracting the illness.

3. Gender: Males are somewhat more likely than women to acquire CLL.

4. Race And Ethnicity: Compared to other racial and ethnic groupings, Caucasians have a higher prevalence of CLL.

5.&nbsp;Chemical Exposure: Chemical exposure has been associated with an increased risk of CLL, including exposure to benzene and Agent Orange.

6. Immune System Weakness: Those with immune systems that are already compromised, such as those with <a href="https://merisehat.pk/disease/hiv-61">HIV</a>/<a href="https://merisehat.pk/disease/aids">AIDS </a>or organ transplant recipients, are more likely to acquire CLL. 
&nbsp;

It is crucial to remember that a person does not necessarily have to have one or more risk factors in order to acquire CLL. In fact, many persons with CLL do not have any recognized risk factors. 
&nbsp;

There are no known methods to stop the onset of chronic lymphocytic leukemia (CLL). However, leading a healthy lifestyle and avoiding certain substances may help lower the chance of developing the illness. 
&nbsp;

There have been several identified possible preventative factors, including:

Maintaining A Nutritious Diet: Consuming a diet high in fruits, vegetables, and whole grains has been linked to a decreased risk of leukemia and other cancers.

Avoiding Exposure To Dangerous Substances: Pesticides and chemicals like benzene have been linked to a higher risk of leukemia. While working with these substances, it&#39;s crucial to take necessary precautions or stay away from them as much as possible.

Stopping The Habit: Smoking has been associated with a higher risk of various cancers, including leukemia.

Maintaining A Healthy Weight: Obesity has been associated with an increased risk of leukemia and other cancers.

Limiting Unneeded Radiation Exposure: Leukemia risk has been related to excessive radiation exposure, such as that from radiation therapy for other cancers. 
&nbsp;

There is no assurance that these preventative measures will particularly stop CLL, even if they may be advantageous for general health. A healthcare professional can identify any potential health risks early on with routine checkups. 
&nbsp;
<quillbot-extension-portal></quillbot-extension-portal>

Key Points

<ul>
	<li>Chronic Lymphocytic Leukemia (CLL) is a type of cancer that affects the blood and bone marrow.</li>
	<li>It is characterized by the accumulation of abnormal white blood cells called lymphocytes.</li>
	<li>CLL is the most common type of leukemia in adults, accounting for approximately one-third of all leukemia cases.</li>
	<li>It primarily affects older adults, with the average age of diagnosis being around 70 years old.</li>
	<li>&nbsp;It is more common in men than women and is more prevalent in certain ethnic groups, including those of Eastern European Jewish descent.</li>
	<li>The exact mechanism of action of CLL is not fully understood, but it is thought to be related to the accumulation and proliferation of abnormal B lymphocytes in the bone marrow, lymph nodes, and other organs of the body.</li>
	<li>Some common signs and symptoms of CLL include enlarged lymph nodes, especially in the neck, armpits, and groin, along with fatigue fever and night sweats and lastly weight loss.</li>
	<li>There are currently no widely accepted screening tests for chronic lymphocytic leukemia (CLL).</li>
	<li>CBC is a test that counts and classifies all of the body&#39;s blood cells, including lymphocytes, which can be overrepresented in CLL.</li>
	<li>Flow cytometry counts the number of abnormal lymphocytes as well as other particular cell types in a blood sample by using antibodies to identify and count them.</li>
	<li>Imaging procedures including computed tomography (CT) scans, magnetic resonance imaging (MRI), and positron emission tomography (PET) scans can be performed to assess the severity of the condition and spot organs or lymph nodes that have enlarged.</li>
	<li>Chemotherapy is a form of cancer treatment that employs chemicals to eradicate cancer cells. Fludarabine, cyclophosphamide, and bendamustine are the most often prescribed chemotherapeutic medications for CLL.</li>
	<li>Patients with advanced-stage CLL who have not responded to conventional therapies may benefit from a stem cell transplant.</li>
	<li>&nbsp;CLL patients may be more prone to infections because of their compromised immune systems.</li>
	<li>Chemotherapy and radiation therapy used to treat CLL can have a number of unfavorable consequences, including exhaustion, nausea, vomiting, hair loss, and lethargy.</li>
	<li>Chemical exposure has been associated with an increased risk of CLL, including exposure to benzene and Agent Orange.</li>
	<li>Consuming a diet high in fruits, vegetables, and whole grains has been linked to a decreased risk of leukemia and other cancers.</li>
	<li>Smoking has been associated with a higher risk of various cancers, including leukemia.</li>
	<li>Leukemia risk has been related to excessive radiation exposure, such as that from radiation therapy for other cancers. 
	&nbsp;</li>
</ul>
<quillbot-extension-portal></quillbot-extension-portal>

[1] National Cancer Institute. (2021). Chronic Lymphocytic Leukemia Treatment (PDQ) – Patient Version.

[2]  American Cancer Society. (2021). Key Statistics for Chronic Lymphocytic Leukemia (CLL).

[3] Miao, Y., Wang, J., & Li, C. (2020). Pathogenesis of chronic lymphocytic leukemia: an update. Journal of Hematology & Oncology, 13(1),

[4] Mukkamalla SKR, Taneja A, Malipeddi D, et al. Chronic Lymphocytic Leukemia. [Updated 2023 Jan 15]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan