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Your Guide to CLL: Understanding Symptoms, Diagnosis, and Treatment

Updated on July 01, 2022
Medically reviewed by
Todd Gersten, M.D.
Article written by
Maureen McNulty

  • Chronic lymphocytic leukemia (CLL) is a common form of leukemia in adults that often does not cause any symptoms until it becomes advanced.
  • Your doctor may recommend starting treatment right away or taking a watch and wait approach.
  • The prognosis (outlook) for CLL depends on many factors, but most people live for at least five more years after diagnosis.

Chronic lymphocytic leukemia is a type of leukemia — a blood cancer — that usually affects older adults. About 20,000 people will be diagnosed with CLL this year in the United States. CLL diagnoses represent more than one-third of leukemia cases in adults, making it the most common type of adult leukemia.

What Is Chronic Lymphocytic Leukemia?

CLL is a form of leukemia that affects the lymphocytes, which are white blood cells that help the body fight infection. CLL develops in the bone marrow — tissue found inside certain bones that produces healthy blood cells. In people with CLL, leukemia cells crowd out the normal blood cells, causing blood cell levels to drop. CLL cells may also collect in the spleen, lymph nodes, and other organs of the lymphatic system, which fights infection and removes waste produced by cells.

Chronic Lymphocytic Leukemia vs. Small Lymphocytic Lymphoma

Doctors consider CLL to be the same disease as small lymphocytic lymphoma (SLL). Both conditions lead to high levels of abnormal lymphocytes and are generally treated the same way. If a person’s leukemia cells are mostly found in the blood and bone marrow, they are diagnosed with CLL. If most of their cancer cells are located in the lymphatic system, they are diagnosed with SLL.

Types of CLL

There are a few different types of lymphocytes. Doctors divide CLL into subtypes depending on which type of lymphoid cell is affected.

According to Cancer.Net, around 95 percent of people with CLL have B-cell CLL, which develops from B lymphocytes. B-cell CLL includes subtypes such as B-cell prolymphocytic leukemia and hairy cell leukemia.

CLL may also develop from T lymphocytes. T-cell CLL subtypes include:

  • T-cell prolymphocytic leukemia
  • Large granular lymphocytic leukemia
  • Adult T-cell leukemia/lymphoma
  • Sezary syndrome

Signs and Symptoms of CLL

Often, CLL does not cause symptoms until the disease becomes more advanced. Many people discover they have CLL only after routine exams or blood tests come back with unusual results. There are several possible signs and symptoms of chronic lymphocytic leukemia:

  • Anemia (low levels of red blood cells) can lead to fatigue, dizziness, and shortness of breath.
  • Leukopenia (low white blood cell counts) and lymphocytosis (high numbers of abnormal white blood cells) may increase the risk of infections.
  • Thrombocytopenia (low platelet count) can cause bleeding problems and easy bruising.
  • Splenomegaly (enlarged spleen) may lead to pain or feelings of fullness in the upper left abdomen.
  • Hepatomegaly (enlarged liver) can cause symptoms including fatigue, abdominal pain, nausea, and vomiting.
  • Lymphadenopathy (swollen lymph nodes) may appear as lumps in the neck, armpit, or groin.
  • Other general symptoms may include fever, chills, night sweats, weight loss, and tiredness. (This group of symptoms is sometimes referred to as B symptoms.)

Many other more common health conditions also cause these symptoms. If you notice any symptoms that could be signs of CLL, talk to your doctor.

Diagnosis of Chronic Lymphocytic Leukemia

Often, doctors will first suspect a person has leukemia after reviewing the results of their routine blood work. An evaluation for the diagnosis will always include collecting your health and symptom history and performing a physical exam, in which a doctor checks for any signs of disease.

Several tests can be helpful when diagnosing CLL. Blood samples can be used for tests like a complete blood count, which measures the levels of different types of blood cells.

The main criteria for a diagnosis of CLL are:

  • B lymphocyte count of above 5,000 per microliter for at least three months
  • Immunophenotyping tests such as flow cytometry, in which a machine “reads” the proteins found on the surface of the leukemia cells
  • Examination of blood cells under a microscope

Additional Testing for CLL Diagnosis and Staging

Blood tests can also be used to determine the levels of proteins like lactate dehydrogenase and beta-2 microglobulin. High levels of these proteins in the blood may be a sign of cancer.

Occasionally, doctors will use bone marrow tests to diagnose CLL. A bone marrow biopsy entails removing a small sample of cells, and a bone marrow aspiration involves removing fluid from inside the bone. Bone marrow tests are not always necessary for diagnosing CLL.

A health care professional may further study cells from blood or bone marrow samples in a lab to learn more about what genes or proteins they contain. This information can help a doctor tell the difference between different types of leukemia and estimate prognosis. Lab tests may include:

  • Cytogenetic tests, such as fluorescence in situ hybridization or gene mutation tests, to identify gene abnormalities within the CLL cells
  • Serum immunoglobulin tests to show whether white blood cells are working correctly or if they are abnormal

If a person is diagnosed with CLL, they may need to undergo imaging tests to determine whether the leukemia is contained within the blood and bone marrow or whether it’s spread to other organs. Doctors may use imaging tests like X-rays, computed tomography (CT) scans, and positron emission tomography (PET) scans. Finding out where cancer cells are located in the body helps doctors determine a person’s stage of cancer.

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CLL Stages

Doctors use leukemia staging information to predict a person’s outlook and determine which treatments may be most helpful. There are a couple of different CLL staging systems. Doctors in the U.S. generally use the Rai system, while European doctors use the Binet system. Both of these systems are based on factors like:

  • Lymphocyte count
  • Levels of red blood cells and platelets
  • Whether a person has an enlarged liver or spleen
  • Whether a person has swollen lymph nodes

Treatment of CLL

Treatments can often keep CLL under control for a long time. When recommending a treatment plan for chronic lymphocytic leukemia, your doctor will take into account factors including:

  • Your age
  • Your overall health
  • Your personal preferences
  • What subtype and stage of CLL you have

Watch and Wait

CLL is a slow-growing leukemia, and some people do not need to start treatment right away. People who use a watch and wait approach have regular follow-up visits with their doctor to monitor their leukemia. If a person begins to develop symptoms, or if tests show that CLL is getting worse, doctors may recommend starting treatment.

Medications for CLL

Treatment of chronic lymphocytic leukemia often includes multiple drugs. Chemotherapies block cancer cells from growing and dividing, which causes those cells to die. Targeted therapy medications, including monoclonal antibodies and kinase inhibitors, block specific genes or proteins that cancer cells rely on to survive. Targeted therapies may be less likely to cause treatment side effects.

In many cases, multiple medications are given together in different combinations to treat CLL. Some treatment options include:

  • FCR, which includes two chemotherapy drugs, fludarabine (Fludara) and cyclophosphamide (Cytoxan), and the targeted therapy drug rituximab (Rituxan)
  • PCR, which includes two chemotherapy drugs, pentostatin (Nipent) and cyclophosphamide, along with rituximab
  • The targeted therapy drugs rituximab, ibrutinib (Imbruvica), acalabrutinib (Calquence), venetoclax (Venclexta), or obinutuzumab (Gazyva), alone or in different combinations with each other
  • The steroid drug prednisone along with rituximab

If CLL relapses (comes back) soon after being treated, doctors may recommend switching to different medications.

Bone Marrow Transplant

People with CLL who are younger, don’t have other major health issues, and are at risk for relapse may be able to undergo allogeneic bone marrow transplant. During this procedure, a person is treated with high-dose chemotherapy. This aggressive treatment kills both leukemia cells and the progenitor cells that produce new blood cells. Following chemotherapy, the person receives new progenitor blood cells from a donor. Those cells divide and make healthy blood cells for the person who received the transplant.

Other Treatment Options

Doctors sometimes recommend other procedures to treat specific CLL symptoms. Among them is radiation therapy, used to treat enlarged lymph nodes. Additionally, if a person with CLL has splenomegaly — an enlarged spleen — their doctor may recommend surgically removing it. People with CLL may also be able to access new treatment options by participating in clinical trials, which are types of research studies.

Outlook for Chronic Lymphocytic Leukemia

In general, 85.7 percent of people with CLL will live for five years or more after being diagnosed. However, your doctor can better estimate your individual CLL prognosis by considering your own personal characteristics. Factors that may lead to a worse prognosis include:

  • Older age
  • Certain gene changes within leukemia cells
  • High levels of cells that contain the proteins ZAP-70 or CD38
  • High levels of beta-2 microglobulin in the blood
  • Quickly growing lymphocytes
  • In some cases, high numbers of prolymphocytes (a type of immature white blood cell)

When estimating your outlook, your doctor may consider a system called the CLL International Prognostic Index (CLL-IPI). This system takes into account specific gene mutations, CLL stage, and age. Your doctor can use the CLL-IPI to give you a risk score that provides information about prognosis and treatment. If you have a lower risk score, you are likely to have a better outcome and may not need to use any treatment. If you are in a high-risk group, you may have a worse prognosis and your doctor may recommend more aggressive treatment.

Feeling Your Best With CLL

Whatever type or stage of CLL you have, and wherever you are in your treatment journey, there are steps you can take at home every day to feel your best and improve your quality of life. Your overall health is an important factor doctors take into account when recommending treatment options. The better your health is in general, the more treatment options you are likely to have.

Talk With Others Who Understand

MyLeukemiaTeam is the social network for people with leukemia and their loved ones. On MyLeukemiaTeam, more than 11,000 members come together to ask questions, give advice, and share their stories with others who understand life with leukemia.

Are you living with chronic lymphocytic leukemia? Share your experiences in the comments below, or start a conversation by posting on your Activities page.

All updates must be accompanied by text or a picture.
Todd Gersten, M.D. is a hematologist-oncologist at the Florida Cancer Specialists & Research Institute in Wellington, Florida. Review provided by VeriMed Healthcare Network. Learn more about him here.
Maureen McNulty studied molecular genetics and English at Ohio State University. Learn more about her here.

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