What is Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)?

Chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) is a type of cancer that results in an increase of mature but malfunctioning B immune cells. CLL/SLL is a condition in which these abnormal B immune cells multiply and accumulate in the body. These abnormal cells, often seen during a blood test, are known as smudge cells. The areas mainly affected by this disease include the blood, spleen, lymph nodes, and bone marrow. Both CLL and SLL come from B immune cells and are effectively the same disease, but they show different symptoms depending on where the abnormal cells are located. CLL is typically the first stage of the disease, where the abnormal cells are present in the blood. Over time, this can progress to SLL, where the abnormal cells are found in the lymph nodes. The term SLL is usually used when this disease process is confined to the lymph nodes.

What Causes Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)?

We’re not entirely sure what causes Chronic Lymphocytic Leukemia (CLL). Genetic factors seem to play a major role, rather than environmental factors. However, there are certain known risk factors for CLL, which include being exposed to specific chemicals through your job, exposure to radiation, and using tobacco.

Studies have indicated a higher risk of CLL amongst farmers, people who work with rubber production, and those exposed to strong solvents like benzene. It’s important to note, though, these links have yet to be confirmed. Interestingly, people who survived atomic bomb explosions haven’t shown a higher rate of CLL, but their risk for other types of leukemia has increased.

Workers, such as uranium miners, who are regularly exposed to both ionizing and non-ionizing radiation, have shown higher incidences of CLL. Smokers and tobacco users also show a significantly higher risk for CLL compared to those who don’t use tobacco.

The Veterans Affairs department recognizes a link between CLL and exposure to Agent Orange or other herbicides during military service.

Risk Factors and Frequency for Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)

Chronic Lymphocytic Leukemia (CLL) is a disease that forms a significant portion of total leukemia cases in the United States, representing about 25 to 30% of them. The American Cancer Society predicts that in 2020, there will be around 21,040 new CLL cases and roughly 4,060 deaths. Globally, 191,000 cases and 61,000 deaths happen due to CLL each year.

Although CLL can affect adults from their 30s, it typically surfaces in adults around the age of 70. Children rarely experience CLL. As people get older, the likelihood of CLL developing increases. Men are somewhat more likely to get CLL than women, but evidence shows that women may experience a more severe form.

The prevalence of CLL varies depending on geographical location and ethnicity. It’s more common in Western adults and shows up more frequently among Caucasians than Asian Pacific Islanders and African Americans. The incidence of CLL mirrors that of the United States in Western countries but is not often seen in Asian countries like China and Japan. Jews of Eastern European descent and Non-Hispanic Whites suffer from CLL more often, while it is least common among Asians. African Americans fall somewhere in between Caucasians and Asian ethnicity groups.

CLL appears to have a genetic component, as it can run in families. Relatives of CLL patients are more at risk for developing the disease. In fact, around 17% of first-degree relatives of CLL patients develop monoclonal B cell lymphocytosis, a precursor of CLL. However, only a small number of people with this precursor condition end up developing CLL.

Signs and Symptoms of Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)

People with Chronic Lymphocytic Leukemia (CLL) often don’t have symptoms at first. CLL is frequently discovered during a routine blood test which shows an abnormally high number of lymphocytes, a type of white blood cell. Around 5 to 10% of people with CLL experience what are called “B” symptoms. These can include:

  • A fever higher than 100.5 degrees Fahrenheit lasting more than two weeks without signs of infection
  • Unexplained weight loss, equal to or more than 10% of body weight over the past six months
  • Intense night sweats, again with no signs of infection
  • Extreme fatigue
  • Feeling full quickly after eating

During a physical exam, between 50 to 90% of people diagnosed with CLL are found to have swollen lymph nodes. These can occur all over the body, but are most common in the neck, above the collarbone, and in the armpit. The swollen nodes feel firm and round, and can be moved around freely under the skin. Between 25 to 55% of people with CLL have a swollen spleen, and between 15 to 25% have a swollen liver. These organs also feel firm but not tender, with a smooth surface on examination.

Doctors will also check the skin as part of the physical exam because skin cancers are a common complication of CLL. Leukemia can cause skin lesions that usually appear on the face in various forms such as small bumps, flat spots, raised areas, sores, blisters, or larger lumps. Sometimes, people with CLL can have over-the-top reactions to insect bites.

In people with CLL, cancerous B cells can build up in the spleen and bone marrow, which can cause the spleen to enlarge. This can cause a drop in red blood cell and platelet numbers, leading to anemia and a tendency to bleed easily. Common symptoms are fatigue and shortness of breath for anemia, and easy bruising and small, pinpoint red spots on the skin (petechiae) for low platelets. Because the B cells are not functioning properly, the body has a harder time making antibodies to fight off infections, putting people with CLL at a higher risk of getting sick.

Testing for Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)

The diagnosis of Chronic Lymphocytic Leukemia (CLL) often begins with a blood test known as a peripheral blood smear. The test looks at the number of a type of blood cell, specifically lymphocytes, and checks for the presence of certain unique cells called smudge cells. If your lymphocyte count is higher than 5000/mcL and smudge cells are found, this can confirm CLL.

On the other hand, patients with Small Lymphocytic Leukemia (SLL) would often show a lymphocyte count of less than 5000/mcL on the same blood test, alongside signs of swollen lymph nodes but without having fewer blood cells than normal. A lymph node biopsy is needed to confirm SLL.

Another procedure called flow cytometry can be performed to confirm the identity of circulating B cells, a type of lymphocyte, proving useful in diagnosing CLL. This test is done on blood and bone marrow samples. This gain further insight into CLL/SLL, specific markers connected with B cells (CD19, CD20, CD21, CD23, and CD24) and a marker linked with T cells called CD5 are identified. Commonly, CLL/SLL will show combined presence of CD5, CD19, and CD23.

Additionally, FISH (a kind of genetic test) is another efficient way to detect genetic abnormalities in patients with CLL/SLL. While not needed expressly for diagnosis, bone marrow aspiration and biopsy are often conducted as a part of an investigative series or before treatment. If more than 30% of the cells in the marrow sample are lymphocytes, it confirms CLL diagnosis. A decrease in lymphocyte counts to less than 30% in treatment is indicative of a complete response.

For examining lymph nodes, a tissue sample is analyzed to check for a particular pattern of lymphocyte distribution known as ” pseudo-follicles”, a sign specific to CLL/SLL patients. One can also use a CT scan to evaluate the degree of swollen lymph nodes and infiltration of organs like the spleen and liver.

The diagnosis of additional complications associated with CLL can also include blood tests. Checks for autoimmune hemolytic anemia usually involve a specific antibody test known as the Coombs test, along with checking for elevated levels of immature red blood cells (reticulocytes), an elevated blood enzyme called LDH, lower levels of a protein called haptoglobin, and a high level of indirect bilirubin (a type of bile pigment). Diagnoses for other complications like pure red cell aplasia and thrombocytopenia can be made through a blood test and bone marrow aspiration and biopsy as well. Some CLL patients may also exhibit decreased levels of immune proteins (hypogammaglobulinemia), high levels of uric acid, and elevated liver enzymes. Two important blood tests in CLL include measuring LDH and beta-2 microglobulin levels – these numbers often reflect the activity level of the disease.

Treatment Options for Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)

Not everyone diagnosed with Chronic Lymphocytic Leukemia (CLL) needs immediate treatment. CLL varies a lot among different people, so some patients may have similar survival rates to healthy people, even without treatment. There are also no current treatments that can cure CLL, unless one goes through a specific type of stem cell transplant. Studies comparing immediate versus delayed treatment have found no significant improvement in long-term survival with starting treatment early.

Treatment is usually recommended if:

  • Patients have symptoms such as fever, night sweats, unintentional weight loss, extreme fatigue, or feeling full quickly.
  • The number of lymphocytes (a type of white blood cell) increases by more than 50% over two months, or if the number of lymphocytes doubles in six months or less.
  • Patient’s cancer progresses rapidly with a large swollen spleen or lymph nodes or with anemia or low platelets levels due to an autoimmune response.
  • There is evidence of bone marrow failing to produce enough healthy cells, resulting in symptoms such as anemia or low platelets level.
  • Patients have recurrent infections.

Treatment for CLL depends on whether the patient has “active disease”. For patients without “active disease”, which include those with early-stage CLL and asymptomatic stage, observation is the standard care without immediate treatment. Patients are closely monitored and based on their health status after a year, a decision on aggressive treatment is made. However, some early-stage CLL patients may require treatment sooner than others.

Alternatively to early-stage asymptomatic CLL, some patients have a localized Small Lymphocytic Lymphoma (SLL) which involves a single lymph node site. The first-line management for these patients is similar to those with asymptomatic CLL, which is observation, but if the disease progresses, radiation therapy is considered.

For patients with active disease, the aim is to alleviate symptoms, prolong remission, and survival. The specific treatment used depends on the patients’ age, general health, genetic features of their tumor, and whether they’ve already started treatment. There are many drugs utilized in the management of CLL, such as Bruton tyrosine kinase inhibitors, Purine analogs, Alkylating agents, Monoclonal antibodies, and PI3K inhibitor. The choice of drug depends on the risk stratification of the tumor along with the patient’s overall fitness.

Potential complications associated with CLL and its treatment include infections and autoantibodies directed against red blood cells and platelets, leading to anemia and low platelet levels. These can be managed with antimicrobials or blood and platelet transfusions.

If CLL becomes resistant to the first round of treatment, it is called relapsed/refractory CLL. Treatment for these cases is determined based on the initial treatment used and the duration of the initial response. There are several possible treatments in this case, including Bruton tyrosine kinase, Alemtuzumab, Venetoclax, and idelalisib medications. In some very aggressive refractory cases, a stem cell transplant, radiation therapy, or CAR T-cell therapy may be considered.

The diagnosis of different types of cancerous blood diseases often includes understanding whether the patient suffers from any of the following conditions:

  • Acute Lymphoblastic Leukemia (ALL), a type of cancer affecting white blood cells.
  • Acute promyelocytic leukemia, a subtype of acute myeloid leukemia, where the body produces too many immature white blood cells.
  • Diffuse large cell lymphoma, a fast-growing type of non-Hodgkin lymphoma.
  • Follicular lymphoma, a type of blood cancer that develops slowly.
  • Hairy cell leukemia, a rare and slow-growing type of leukemia.
  • Lymphoblastic lymphoma, a rare non-Hodgkin lymphoma.
  • Mantle cell lymphoma, a non-Hodgkin lymphoma type that grows quickly.
  • Non-Hodgkin lymphoma, a group of blood cancers that includes all types of lymphoma, except for Hodgkin’s lymphomas.
  • Monoclonal B-cell lymphocytosis (MBL), a condition where the body has too many white blood cells, but without causing any symptoms.
  • Prolymphocytic lymphoma (PLL), a rare type of blood cancer.
  • Lymphoplasmacytic lymphoma, a rare type of non-Hodgkin lymphoma that affects the body’s ability to fight infection.
  • Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) can sometimes transform (‘Richter transformation’) into a more serious condition, either into Diffuse Large B-Cell Lymphoma or Hodgkin’s Lymphoma.

Each of these conditions has a different impact on patients’ health and requires specific types of treatment, so a precise diagnosis is very important.

What to expect with Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)

For people diagnosed with Chronic Lymphocytic Leukemia (CLL), their survival time can range anywhere between two to over 20 years, with the average survival period being 10 years. Some patients don’t require any treatment immediately and can survive between 5 to 20 years.

An important factor to consider is the lymphocyte doubling time, meaning how fast the number of lymphocytes (a type of white blood cell) in the blood doubles. Faster doubling times of less than 12 months are often seen in more aggressive cases of untreated CLL.

There are certain factors that often indicate a better prognosis, such as variations in the Ig heavy chain (a part of the immune system), a specific deletion of genetic material known as 13 q deletion, low ZAP-70, and low CD38 levels on a test known as flow cytometry.

On the flip side, there are factors that often signal a worse prognosis. These include certain genetic abnormalities such as 17p and 11q deletions. Patients with widespread lymph node involvement, enlarged liver and spleen, anemia, and low platelet count, generally have a worse prognosis.

There are tools that help predict the outcome for patients with CLL; one such widely used tool is the CLL-IPI or the International Prognostic Index for CLL.

Possible Complications When Diagnosed with Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)

People with CLL (Chronic Lymphocytic Leukemia) may have several complications. These could include becoming more prone to infections, especially infections of the respiratory tract. There’s also the risk of the illness advancing to a more serious type of lymphoma called diffuse large B-cell lymphoma (also known as Richter syndrome), and an increased risk of getting other types of cancer such as skin, lung, and gastrointestinal tract cancers. Another complication could be issues with the immune system attacking the body’s own red blood cells or platelets, but this happens very rarely.

Common Complications:

  • Increased susceptibility to infections, particularly of the respiratory tract
  • Progression to diffuse large B-cell lymphoma (Richter syndrome)
  • An elevated risk of other malignancies (e.g., cancers of the skin, lungs, and GI tract)
  • Immune system issues, where the immune system attacks red blood cells or platelets (though rare)

Preventing Chronic Lymphocytic Leukemia (Chronic Cancer of White Blood Cells Called Lymphocytes)

People with CLL (chronic lymphocytic leukemia) need to comprehend what their illness entails, the progression stages, where they are in that progression, the treatments on offer, and their expected outcome based on the specific type of their illness. It’s crucial that patients and their families are included in every decision about treatment and management. In later stages of the illness, it might be beneficial to seek emotional and psychological support from trained mental health professionals.

Frequently asked questions

Chronic Lymphocytic Leukemia (CLL) is a type of cancer that results in an increase of malfunctioning B immune cells.

Chronic Lymphocytic Leukemia (CLL) represents about 25 to 30% of total leukemia cases in the United States.

Signs and symptoms of Chronic Lymphocytic Leukemia (CLL) include: - Often no symptoms at first, with CLL frequently being discovered during a routine blood test that shows an abnormally high number of lymphocytes. - "B" symptoms, which occur in 5 to 10% of people with CLL, can include: - A fever higher than 100.5 degrees Fahrenheit lasting more than two weeks without signs of infection. - Unexplained weight loss, equal to or more than 10% of body weight over the past six months. - Intense night sweats, again with no signs of infection. - Extreme fatigue. - Feeling full quickly after eating. - Swollen lymph nodes are found in 50 to 90% of people diagnosed with CLL. These can occur all over the body but are most common in the neck, above the collarbone, and in the armpit. The swollen nodes feel firm and round and can be moved around freely under the skin. - Between 25 to 55% of people with CLL have a swollen spleen, and between 15 to 25% have a swollen liver. These organs also feel firm but not tender, with a smooth surface on examination. - Skin cancers are a common complication of CLL, so doctors will check the skin during a physical exam. Skin lesions can appear on the face in various forms such as small bumps, flat spots, raised areas, sores, blisters, or larger lumps. Over-the-top reactions to insect bites can also occur. - Cancerous B cells can build up in the spleen and bone marrow, causing the spleen to enlarge. This can lead to a drop in red blood cell and platelet numbers, resulting in anemia and a tendency to bleed easily. Symptoms of anemia include fatigue and shortness of breath, while low platelets can cause easy bruising and small, pinpoint red spots on the skin (petechiae). - Due to the improper functioning of B cells, people with CLL have a higher risk of getting sick as their body has a harder time making antibodies to fight off infections.

The exact cause of Chronic Lymphocytic Leukemia (CLL) is not entirely known, but genetic factors seem to play a major role. Other risk factors include exposure to specific chemicals through your job, exposure to radiation, and using tobacco.

The doctor needs to rule out the following conditions when diagnosing Chronic Lymphocytic Leukemia (CLL): 1. Acute Lymphoblastic Leukemia (ALL) 2. Acute promyelocytic leukemia 3. Diffuse large cell lymphoma 4. Follicular lymphoma 5. Hairy cell leukemia 6. Lymphoblastic lymphoma 7. Mantle cell lymphoma 8. Non-Hodgkin lymphoma 9. Monoclonal B-cell lymphocytosis (MBL) 10. Prolymphocytic lymphoma (PLL) 11. Lymphoplasmacytic lymphoma 12. Richter transformation into Diffuse Large B-Cell Lymphoma or Hodgkin's Lymphoma.

The types of tests needed for Chronic Lymphocytic Leukemia (CLL) include: 1. Peripheral blood smear: This test looks at the number of lymphocytes and checks for the presence of smudge cells. 2. Flow cytometry: This test is done on blood and bone marrow samples to confirm the identity of circulating B cells and identify specific markers associated with CLL/SLL. 3. FISH (Fluorescence In Situ Hybridization): This genetic test detects genetic abnormalities in patients with CLL/SLL. 4. Lymph node biopsy: A tissue sample from lymph nodes is analyzed to check for specific patterns of lymphocyte distribution. 5. CT scan: This imaging test evaluates the degree of swollen lymph nodes and infiltration of organs like the spleen and liver. 6. Blood tests: These tests can check for complications associated with CLL, such as autoimmune hemolytic anemia, pure red cell aplasia, thrombocytopenia, and decreased levels of immune proteins. 7. Bone marrow aspiration and biopsy: These tests may be conducted as part of an investigative series or before treatment to confirm CLL diagnosis and assess treatment response.

Treatment for Chronic Lymphocytic Leukemia (CLL) depends on whether the patient has "active disease" or not. For patients without "active disease", which include those with early-stage CLL and asymptomatic stage, observation is the standard care without immediate treatment. These patients are closely monitored, and a decision on aggressive treatment is made based on their health status after a year. However, some early-stage CLL patients may require treatment sooner than others. For patients with active disease, the aim is to alleviate symptoms, prolong remission, and survival. The specific treatment used depends on the patients’ age, general health, genetic features of their tumor, and whether they've already started treatment. There are many drugs utilized in the management of CLL, such as Bruton tyrosine kinase inhibitors, Purine analogs, Alkylating agents, Monoclonal antibodies, and PI3K inhibitor. The choice of drug depends on the risk stratification of the tumor along with the patient's overall fitness. In cases of relapsed/refractory CLL, treatment is determined based on the initial treatment used and the duration of the initial response, and may include medications like Bruton tyrosine kinase, Alemtuzumab, Venetoclax, and idelalisib. In some very aggressive refractory cases, a stem cell transplant, radiation therapy, or CAR T-cell therapy may be considered.

When treating Chronic Lymphocytic Leukemia (CLL), there can be several side effects and complications. These include: - Increased susceptibility to infections, particularly of the respiratory tract. - Progression to diffuse large B-cell lymphoma (Richter syndrome). - An elevated risk of other malignancies, such as cancers of the skin, lungs, and gastrointestinal tract. - Immune system issues, where the immune system attacks red blood cells or platelets (though this is rare).

The prognosis for Chronic Lymphocytic Leukemia (CLL) can vary depending on several factors. The average survival period for CLL is 10 years, but the range can be anywhere from two to over 20 years. Some patients may not require immediate treatment and can survive between 5 to 20 years. Factors that indicate a better prognosis include variations in the Ig heavy chain, a specific deletion of genetic material known as 13 q deletion, low ZAP-70, and low CD38 levels. On the other hand, factors that signal a worse prognosis include certain genetic abnormalities such as 17p and 11q deletions, widespread lymph node involvement, enlarged liver and spleen, anemia, and low platelet count.

An oncologist or hematologist is the type of doctor you should see for Chronic Lymphocytic Leukemia (CLL).

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